Page Menu ACNE ALOPECIA BULLOUS PEMPHIGOID DARIER’S DISEASE ECTODERMAL DYSPLASIA ECZEMA EHLERS-DANLOS SYNDROME EPIDERMOLYSIS BULLOSA HERPES VIRUSES HIDRADENITIS HYPOMELANOSIS OF ITO ICHTHYOSIS LEG ULCERS & PRESSURE SORES LICHEN SCLEROSUS LUPUS LYMPHOEDEMA NEUROFIBROMATOSIS PEMPHIGUS VULGARIS PSEUDOXANTHOMA ELASTICUM PSORIASIS PSORIATIC ARTHRITIS RAYNAUD’S & SCLERODERMA ROSACEA SHINGLES & CHICKENPOX SKIN CANCER TUBEROUS SCLEROSIS VITILIGO XERODERMA PIGMENTOSUM Please wait until the page is loaded, then choose a category from the above list, this will take you to the correct section of the page. ACNE Acne is an inflammatory skin condition, commonly affecting the face, chest and back. It is one of the most common skin conditions, affecting up to 80-90% of adolescents. Acne may also occur during infancy due to the activation of sebaceous glands by maternal hormones in-utero. It can be a persistent problem, although it spontaneously resolves after a period of 4-5 years in about 70% of people. Approximately 30% of people continue with their acne into adult life. The incidence of acne at the age of 40 is reported to be 1% in men and 5% in women. Causes Acne is caused by the overactivity of the sebaceous (oil) glands. Sebaceous glands are active due to the hormone testosterone. Testosterone is produced in men from the testes and in women from the ovaries and the adrenal gland. Although testosterone levels are usually normal in people who develop acne, the sebaceous glands are overly sensitive to the hormone. Over production of oil leads to a greasy feel to the skin. In addition, there is a change in the growth of the cells lining the follicular canal (pore). Instead of dislodging normally and being carried away by the oil (sebum) when they die, these cells become sticky and stick to the inner surface of the gland and gradually build up a partial blockage. Symptoms The partial blockage of the pore is called a microcomedone and is the precursor of all inflammatory and non-inflammatory spots in acne. Bacteria start to multiply within the blockage, which leads to inflammatory lesions or red papules. As part of the healing process, the body recruits white blood cells which destroy the bacteria but lead to the formation of pus, leading to comedones (blackheads), small pimples, large pustules, or painful swollen cysts. There are many myths surrounding the causes of acne, which include: only teenage spots - you'll grow out of it. eating too much chocolate and too many chips. not keeping your skin clean enough. Acne can be a devastating psychological disease commonly affecting the face, neck, chest, shoulders and back. The latest survey by the Acne Support Group shows that 12% of people with acne feel suicidal as a result of having the condition. Whilst it is a very common skin condition, it need not be left untreated and leave a person feeling so desperate that they want to take their own life. Treatments There are many treatments available, which need to be given at an early stage to prevent scarring. Treatments are generally longterm, however with encouragement and support a person can improve their skin substantially. First line treatment for acne includes creams or gels containing the active ingredient benzoyl peroxide, which is available from chemists. Salicylic acid is also available from chemist shops and can be effective in comedonal (blackhead) type acne. Antibiotics are a very popular treatment for acne. These help to reduce inflammation, whilst killing the bacteria. Other treatments include preparations containing the hormone therapy cyproterone acetate (females only). For those who have not responded to systemic and topical therapies, isotretinoin, which is a synthetic vitamin A, can be very useful. This is a hospital-only drug and can achieve up to 95% positive results in patients. There are many potential side effects from this drug and hence the patient will need monitoring by their dermatologist. Generally, treatments need to be taken for two months before any improvement is noticed (the ‘two month rule’). For further information contact: Acne Support Group, PO Box 9, Newquay TR9 6WG Reg. Charity No. 1026654. Tel: 0870 870 2263 Email: alison.dudley@btopenworld.com Website: www.stopspots.org The Acne Support Group provides information and support to those people affected by acne and those people affected by rosacea. Information and services available to members include: a comprehensive information pack. confidential advice. a lively and informative newsletter. Return to the Top of Page ALOPECIA Alopecia is a generic medical term for all forms of hair loss, from the patchy baldness of alopecia areata, which in some cases becomes total loss, to the diffuse thinning and ‘male pattern baldness’ of alopecia androgenetica. Hair loss can be sudden, frightening and severe. Although it is not life threatening or even physically painful, alopecia causes a high level of psychological and emotional suffering. A survey by Hairline International of women who had lost, or were losing, their hair found that 76% felt less of a woman; 40% said that marriages (or long term partnerships) had suffered and of these many had broken up; and 63% said they had been forced to compromise a career. In many cases, alopecia patients experience severe depression. A large proportion of the respondents (43%) had considered suicide. In addition, patients often face cruel jokes from others and find that some healthprofessionals dismiss the condition as superficial. Types and Symptoms Alopecia areata (patchy baldness) affects men, women and children. It often begins at puberty. This scalp disease usually starts with a tiny circumscribed patch of baldness. Other patches may follow and as one patch re-grows hair frequently falls out in another. Alopecia areata frequently spreads very quickly, sometimes throughout the scalp. The affected hair follicles slow down production, become very small and often grow no hair that is visible above the surface for months or years. But the follicles normally remain alive and are ready to resume normal hair production whenever they receive the appropriate signal or ‘trigger’. In a third of all cases patients will have only one small patch of baldness. The hair re-grows spontaneously and they never suffer a further episode. Two thirds of patients suffer the patchy baldness of alopecia areata throughout their lives. Alopecia areata can develop into total loss of scalp hair (alopecia totalis) or the loss of all body hair including the scalp - alopecia universalis - which occurs in about one fifth of cases. Alopecia androgenetica (male pattern baldness) - a large number of women suffer from thinning hair. In a woman this can be the female version of alopecia androgenetica, the natural balding of ageing. It often occurs after the menopause, but is also prevalent in younger women who are genetically predisposed to the condition. It can manifest itself when triggered by such factors as eating disorders or an over-sensitivity to the progestogens contained in some types of contraceptive pill. Male pattern baldness often causes a great deal of distress, particularly in men whose work brings them into contact with the public. It can cause a marked fall in self-esteem. Telogen effluvium - the ‘human moult’ hair loss occurs after the body has suffered severe trauma. It can occur after a high fever, childbirth or extreme shock. Hair loss related to medication -Some drugs prescribed for other conditions can cause hair thinning. Including some psychiatric drugs (eg. anti-depressants) and chemotherapy treatment. Self-inflicted hair loss -Trichotillomania is an obsessional compulsive disorder in which many patients pull out their own hair. Causes Alopecia areata is believed to be an autoimmune disease in which the body rejects the hair follicle as foreign. Atopic syndrome often plays a part and alopecia areata frequently occurs in patients who have experienced asthma or eczema from birth. Other factors, which can be involved in its onset, are thyroid conditions, anaemia, vaccinations and stress. It can also be a family problem. One in five patients can recall a relative with a similar condition. Many younger women develop alopecia androgenetica, because they have an over-sensitivity to the androgens (male hormones) in the blood. When they have this pre-disposed sensitivity, contraceptive pills containing progestogen can exacerbate the problem. Hormone replacement therapy (HRT) has similar components to the pill and many women find that their hair becomes thinner during this treatment. The stress of modern life is a common causative agent. Treatments Re-growth success is not always easy to achieve and doctors are cautious of raising false hope by offering treatment. For alopecia areata, and its related conditions, re-growth success has been achieved by the use of a combination therapy consisting of topical steroid creams plus topical minoxidil, systemic steroids (such as prednisolone) and, occasionally, zinc. The administration of powerful steroids can sometimes be enough to 'kick-start' the hair into re-growth, although doctors urge caution and careful monitoring for side-effects. In severe cases and those of total loss, the phenol derivative diphencyprone can be successful. In less severe cases of both Alopecia Androgenetica and Alopecia Areata, topical minoxidil alone can promote re-growth. Anti-androgen drugs such as cyproterone acetate can also help in alopecia androgenetica. Finasteride, the drug for the treatment of enlarged male prostate, has recently been licensed for the treatment of hair loss in men. For further information contact: Hairline International, The Alopecia Patients’ Society, Lyons Court, 1668 High Street, Knowle, West Midlands, B93 OLY. The Hair Trust Reg. Charity No. 1056204. Tel: 01564 775281. Fax: 01564 782270. For information on membership please enclose an A4 SAE. Website: www.hairlineinternational.co.uk Hairline International is a national support network for those who have lost, or are losing, their hair and supports all alopecia patients whatever their type of hair loss. The organisation: maintains a network of contacts to share experiences. advises on suitable prostheses and available experts. campaigns to raise public awareness. Return to the Top of Page BULLOUS PEMPHIGOID Bullous pemphigoid is a rare, blistering skin disease which can occur anywhere on the skin, but is more commonly found on the folds of the skin, particularly the groin area and the armpits. The disease is more prevalent in the elderly, but an increasing number of people are being diagnosed when in their early fifties. The average age of onset for the disease is between 65 and 75. The condition affects males and females in equal proportion and is rarely life-threatening. In approximately 30% of people the condition burns itself out after a number of years, although a recurrence can occur in later life. Causes Bullous pemphigoid is an autoimmune disease, where an immune response is triggered to the body’s own skin cells. Separation occurs between the epithelial and dermal layers of the skin. The reason for this immune response remains the subject of research. The condition is not contagious and may not be passed on by skin contact. Symptoms During a flare-up a non-specific rash first develops on the folds of the limbs. The skin becomes inflamed (erythematous) and very itchy (pruritic). The patient reports feeling very tired and agitated. The disease is at times very debilitating and distressing, with simple tasks becoming a real effort to perform. The stress of the disease can exacerbate the condition. These changes can resemble other skin diseases, such as urticaria, erythema multiforme and dermatitis herpetiformis. Consequently GPs often do not recognise these signs as the early symptoms of bullous pemphigoid. Following this (prodromal) phase, fluid-filled blisters develop. A diagnosis of the condition is confirmed by examination of a blister by skin biopsy. The prodromal phase can last from a single week to several months. Although the condition sometimes remains localised, it is more common for dense bullae to progressively cover both inflamed and normal skin over the majority of the body area. The skin feels hot, itchy and very tender. The lesions, which can be several centimetres in diameter, are particularly concentrated in body folds. Lesions may also develop in the mouth and other mucosal membranes. Treatments When a widespread blistering flare-up occurs, the patient is usually admitted to hospital. As yet there is no cure for bullous pemphigoid, but the condition can be controlled using potent medications. Depending on the severity of the blistering and the patient’s general health, it can be treated very successfully using steroids and/or immunosuppressants. A large initial dosage of steroids is administered during a flare-up, in order to bring the blistering under control. These treatments can have harmful side effects, and over the subsequent weeks and months the dosage is gradually reduced until the lowest efficacious dose is attained which will control the condition. A low dosage of the drug treatment is administered for several months or years in order to maintain the condition. It is important to be aware of the risk of infection to the skin, especially when blisters are broken. An antibacterial ointment is routinely applied to the skin for this purpose. In between flares, although the majority of people experience no outward evidence of the disease, it is advisable to keep the skin well hydrated using emollients (creams, lotions and bath oils). Some people find bathing with a non-scented oil very soothing, whilst others have reported an intolerance to any bathing. Some people report adverse reactions to strong sunlight. Although exposure to ultraviolet (UV) light has not been proven to precipitate or to exacerbate the condition, exposing the skin to strong sunlight is inadvisable as the use of steroid creams will have thinned the skin, thereby increasing the risk of skin damage due to sun exposure. For further information contact: Bullous Pemphigoid Support Group, 17 Barley Mount, Redhills, Exeter, EX4 1RP. The Bullous Pemphigoid Support Group aims to: offer support to people with bullous pemphigoid. establish commonality between experiences, in order to gain a greater understanding of the causes of the disease and treatment regimens. Return to the Top of Page DARIER'S DISEASE This disease was first described in 1889 by Jean Darier, a French dermatologist. It is a rare condition characterised by itchy, warty bumps often involving the chest, neck and upper back. The condition can affect both men and women. Symptoms The first signs of the condition usually appear between the ages of 6 and 20, but may begin when people are older or, rarely, younger. The severity of the condition varies enormously and is unpredictable. Small brownish, rough topped bumps develop on the skin. Some patients have scattered spots which cause very little trouble, but in others the disease is more pronounced. The chest, neck or upper back are often involved at the beginning, but warty bumps may occur on any part of the body. It is unusual for people to have much trouble on the face, except for the skin on the forehead near the hairline. The scalp and skin around the ears may be scaly and itchy and most people notice some small spots in the armpits, the groin or, in women, under the breasts. The fingernails are usually affected. They tend to be rather fragile, split easily and look as if they have been bitten or appear dirty. There may be very obvious long red or white lines running the length of the nails. Nail changes or flat warts on the backs of the hands are often present in childhood, before other symptoms appear. Pits or small corns occur on the palms of the hands and less often the soles of the feet. Occasionally there may be small spots inside the mouth, which give the roof of the mouth a rough feeling. Although the condition is not infectious or contagious, people with Darier’s disease show an increased susceptibility to herpes simplex infections, which exacerbates the symptoms of the condition. Though the skin may be clean, affected areas may smell unpleasant, due to bacterial growth in the rash. The condition is aggravated in the summer months due to an inherent photosensitivity, and through stress. Causes It is a dominantly inherited condition. There is a 1-in-2 chance that each child of an affected parent will inherit the disease. The condition is caused by a change (mutation) in a gene on chromosome 12. This gene makes a protein found within keratinocytes called SERCA2. The SERCA2 protein acts as a ‘calcium pump’. Cells use calcium to produce signals that control the complicated ‘machinery’ inside the cell. In Darier’s disease we believe signalling is faulty in the skin because the calcium pumps do not work properly. This leads to breakdown in the normal bonding of skin cells. The skin becomes scaly, lumpy and may blister. Treatments Retinoids (Vitamin A derivatives) are taken orally and improve the overall condition of the skin, by reducing its lumpiness and scaling, in most patients. Care must be exercised when prescribing to sexually active women, as retinoids could damage an unborn child. Therefore retinoids are only prescribed to sexually active women who have been sterilised, or who are using an effective contraceptive. Retinoids also cause the drying of lips, eyes and nose and patients with mild forms of the disease often decide to live with the symptoms of the condition rather than these side effects. Itching (pruritus) is very common. Emollients containing an anti-pruritic may relieve some of the irritation. More severe pruritus can be controlled with a corticosteroid cream, containing an antibiotic to prevent skin infection. As mentioned the affected skin may smell unpleasant, particularly in moist areas, due to a bacterial growth in the rash. This does not mean that the skin is dirty. Careful washing is important, and antiseptic solutions for the bath, as well as creams or antibiotics may help. The condition can be exacerbated by heat, sweating and, wool or nylon clothing. Some patients find that sunlight causes the skin to flare up, whilst some women find the condition worsens around the time of their period. In a quarter of patients the condition improves as they get older. Most people lead full and active lives, with less than a quarter needing time off work or school because of the condition. For further information contact: Darier's Disease Support Group (DARDIS), PO Box 36, Milford Haven, Dyfed, SA73 3YF. Tel: 01646 695055. Website: http://www.dariers.8m.com The Darier's Disease Support Group provides information and support to people with Darier's disease, with the motto: Do not give up hope. Awareness to others. Readiness to smile. ADefinite goal. Isolation no longer. Speak and be heard. Return to the Top of Page ECTODERMAL DYSPLASIA A Ectodermal Dysplasia (ED) is not a single disorder, but a group of closely related conditions of which more than 150 different syndromes have been identified. The Ectodermal Dysplasias (EDs) are genetic disorders affecting the development or function of the teeth, hair, nails and sweat glands. Depending on the particular syndrome ED can also affect the skin, the lens or retina of the eye, parts of the inner ear, the development of fingers and toes, the nerves and other parts of the body. Causes The ectodermal dysplasias are caused by altered genes. The altered genes may be inherited or the normal genes may become defective (mutate) at the time of conception. The chances for parents to have affected children depend on the type of ED that exists in the family. It is important to remember that a person cannot chose or modify the genes that he or she has, and that events of pregnancy do not change the genes. Thus, parents who have a child with ED should not think that they did anything to cause the defective gene and should not blame themselves for its existence. The inheritance patterns are variable according to the specific type of ED. Patterns include spontaneous mutations, autosomal dominant, autosomal recessive, X-linked dominant and X-linked recessive. When questions of a diagnosis exist, the expertise of a geneticist or other doctor with experience with the EDs is strongly recommended. Genetic counselling is available for families. Symptoms Each syndrome usually involves a different combination of symptoms, which can range from mild to severe, such as: It is important to remember that not all individuals affected by the EDs will have physical features that fit the description of a specific syndrome. Absence or abnormality of hair growth Absence or malformation of some or all teeth Inability to perspire, which causes overheating Impairment or loss of hearing or vision Frequent infections due to immune system deficiencies or, in some cases, the inability of cracked or eroded skin to keep out disease-causing bacteria Absence or malformation of some fingers or toes Cleft lip and/or palate Irregular skin pigmentation. In addition to the above they may have: Cleft lip and/or palate Sensitivity to light Respiratory problems A lack of breast development A host of other challenges It is important to remember that not all individuals affected by the EDs will have physical features that fit the description of a specific syndrome. There may be a great deal of variation in the physical appearance of the same type of ED from one affected person to the next. It is also conceivable for a person to have a type of ED that has not been described yet. Nonetheless, the EDs share certain features, an understanding of which makes it possible to appreciate the ramifications for most affected individuals and allows everyone involved to respond appropriately to the individual’s needs. Treatments Individuals affected by ED face a lifetime of special needs which may include: Dentures at a young age with frequent adjustments and replacements Special diets to meet dental/nutritional needs Air conditioned environments Wigs to conceal hair and scalp conditions Carrier identification testing Protective devices from direct sunlight Osseointegrated dental implants Respiratory therapies For further information contact: The Ectodermal Dysplasia Society, 108 Charlton Lane, Cheltenham, Glos. GL53 9EA. Tel: 01242 261332 Reg. Charity no. 1089135 Email: diana@ectodermaldysplasia.org Website: www.ectodermaldysplasia.org Contact: Mrs. Diana Perry The Ectodermal Dysplasia Society aims to: obtain answers from Medical professionals to members’ specific questions support families when they approach organisations such as Local Authorities, Social Services, etc., by putting together a personal report explaining very simply how ED affects them liaise with Head Teachers, Health Authorities and medical professionals help families get the right care for their child in schools, such as full or part time Carers, fans, air-conditioning, etc. help more families obtain Disability Living Allowance, Disability Carers Allowance, etc. support members in their fundraising put people in touch with each other if requested find pen pals for the younger members help families obtain information regarding ante-natal testing put the Society on the databases of Health Authorities, NHS Trusts, Health organisations, etc. The Ectodermal Dysplasia Society has their own Medical Advisory Board consisting of 12 professionals from various clinical fields. Return to the Top of Page ECZEMA The word eczema comes from the Greek and means ‘to boil over’. The main features of eczema are dry, itching, red and inflamed skin. The words eczema and dermatitis mean the same thing. Eczema affects about one in every ten people in the United Kingdom and can be mild, moderate or severe. Eczema can be a disruptive and distressing condition and can affect all areas of personal and family life. Types, Symptoms and Causes Atopic eczema – This is the most common type of eczema. It usually starts in babies and young children and is thought to affect up to one in every five children. The main features are itching, redness, and inflammation. Dry, scaling skin is often seen in the creases of legs, wrists and neck as well as on the face and forehead. If the skin is weeping and crusting the skin may be infected. Atopic eczema is an inherited condition, linked to asthma and hayfever. It is thought that people with atopic conditions are sensitive to things found in their environment (allergens) which people that are not atopic find harmless. Allergens may affect the skin by direct contact, or by being breathed in or swallowed. Eczema is not contagious – it cannot be caught from someone else. Many people have mild to moderate eczema, which can be successfully managed. However, some people do have severe eczema, which may sometimes need hospital treatment. Three quarters of children with atopic eczema grow out of it by the time they reach their teenage years. Contact dermatitis – There are two types of contact dermatitis: allergic and irritant. Both types have similar symptoms, though the hands are most often affected. It is sometimes referred to as occupational dermatitis due to the impact it can have on a person’s occupation: Allergic contact dermatitis – This tends to appear where the skin is in direct contact with something, for example, the earlobes in nickel allergy (if wearing earrings). It is caused when the immune system overreacts to a substance that would normally be considered harmless, and creates an allergic response. Common allergens include nickel, chromates, cobalt, rubber, formaldehyde, colophony, acrylates, epoxy, fragrances, plants, hair perm solutions, hair bleaching preparations, flour and garlic. It can be a painful and disabling condition with skin which is often dry, red, split, cracked, weeping, fluid-filled and intensely itchy, sore and stinging. If the condition is related to the person’s work, a change of career is sometimes necessary. Jobs that are at high risk include hairdressing, catering, cleaning, construction, engineering, printing, health care, agriculture and horticulture. Irritant contact dermatitis – This has virtually the same signs and symptoms as allergic contact dermatitis but is caused by repeated contact with an irritant substance such as diluted acids, diluted alkalis, solvents, soaps, detergents, metallic salts, cement, resins and cutting fluids. The most common occupations at risk of irritant contact dermatitis are those that involve wet work, for example, chefs, bakers, bartenders, caterers, cleaners, hairdressers, metal workers, surgical nurses, printers, solderers, fishermen and construction workers. Seborrhoeic eczema – This can occur in adults, children and babies. In babies it is often associated with ‘cradle cap’. It usually starts on the scalp as dandruff that can progress to redness, irritation and scaling which can spread to the face and skin creases. It is a reaction to the increased production of pityrosporum ovale, a yeast that occurs normally on the skin in those areas which generally produce a lot of oil such as scalp, face and chest and back in men. Candida (which causes thrush) can also be found on the skin of people with seborrhoeic eczema and can make the condition worse. Gravitational eczema – Also known as varicose or stasis eczema, this type appears on the lower legs and generally affects people in later life, particularly women. It is related to poor blood circulation and high blood pressure. Special care needs to be taken to make sure that legs are not knocked as the skin can become thin, fragile, shiny and flaky which can lead to leg ulcers. Treatments The main treatment for eczema is emollients (moisturisers) and an explanation of the condition and its treatments. Other treatments for mild to moderate eczema might include topical corticosteroids (applied to the skin), antibiotics, and bandaging. People with eczema might also be given advice on how to avoid allergens, the ‘triggers’ that make their eczema worse. Some people also find complementary medicines useful to treat their eczema. Severe eczema might be treated with stronger topical corticosteroids, ultra-violet light therapy, drugs which suppress the immune system, such as cyclosporin, and oral steroids taken by mouth. New treatments, known as topical immunomodulators, such as tacrolimus and pimecrolimus, are now available for people with atopic eczema. Tacrolimus is licenced for patients who do not respond adequately to topical corticosteroids. Pimecrolimus is used to try and prevent flares. For further information contact: National Eczema Society, Hill House, Highgate Hill, London, N19 5NA. Reg. Charity No. 1009671 A company limited by guarantee registered in England No. 2685083 Tel: 020 7281 3553 Fax: 020 7281 6395 Eczema Information Line: 0870 241 3604, email: helpline@eczema.org Websites: www.eczema.org Professional members' site: www.eczemapro.org The National Eczema Society is dedicated to meeting the needs of people with eczema and their families by: providing support and information on the disease and its management producing publications on eczema and its management providing information for health care professionals and a professional membership scheme funding research into causes of and treatments for eczema Return to the Top of Page EHLERS-DANLOS SYNDROME Ehlers-Danlos Syndrome (EDS) is a group of heritable disorders of connective tissue, characterised by skin extensibility, joint hypermobility and tissue fragility. There are different types of EDS and these were re-classified in 1997 into six major types, classified according to their symptoms and signs with each type running true in a family. Thus an individual with one type will not have a child with a different type. EDS is known to affect both males and females of all races and ethnic backgrounds, with an estimated prevalence of 1 in 5000. Causes EDS is caused by a defect in the collagen (connective tissue), which is the main building block in the body. Collagen provides strength for the different parts of the body. Some types are firm to give support, others are elastic to allow movement and strength, and still others resemble glue which bind proteins together. Consequently, defects in collagen can produce many problems. Symptoms Diagnosis is based on the presenting symptoms and family history. Diagnosis can be delayed, or overlooked, in some cases as they do not fit conveniently into a specific type. A skin biopsy may be taken to study the connective tissue. Specific tests are available for certain types of EDS. Relating to the skin Hyperextensibility. Stretchy skin characterises all EDS except for the Vascular Type (type lV), which has noticeably translucent skin with visible veins. When the skin is over-stretched it still retains normal elastic recoil and snaps back once released. This is best tested at the neck, elbows or knees. Fragility. Easy splitting of the skin is particularly common in Classical Type (Types l and ll). Gaping, ‘fish-mouth’ or ‘cigarette paper’ scars follow minimal trauma over sites of bony prominence and, areas prone to trauma such as the forehead, chin, elbows, knees and shins. Epicanthic folds. These are additional symmetrical folds of skin at the inner aspects of the eyes producing apparent broadening of the nose. Molluscoid pseudotumours. These are firm, fibrous lumps measuring up to 2-3 cm that develop over pressure points such as the elbows and knees. Spheroids. Approximately one third of affected individuals describe small, firm nodules like ‘ball-bearings’ just beneath the skin (subcutis). These consist of fibrotic and calcified fat, which overlays bony areas such as the shins. Piezogenic papules. These small, soft, skin-coloured lumps appear on the side of the heel when standing and disappear when the foot is elevated. Although usually symptom-less they can occasionally be painful. Varicose veins. These are more common in many types of EDS. Relating to the joints Hypermobility is assessed using the Beighton scale. A score of 5/9 or higher defines hypermobility. Dislocation and partial dislocation; this is common due to unstable joints. Chronic joint and limb pain. Pain is common even when skeletal X-rays are normal. Bruising and haematomas Easy bruising, at sites of trauma, accompanies most forms of EDS. This implies increased fragility of blood capillaries and poor structural integrity of the skin. When bruising presents in a child it may be incorrectly attributed to non-accidental injury. Mitral valve prolapse This is quite common and should be diagnosed by echocardiography, CT scan or magnetic resonance imaging (MRI). Less common features Arterial/uterine/intestinal rupture due to tissue fragility. Hernias are also relatively common. Scoliosis (curvature of the spine) may be present at birth or can develop in later life. Gum disease. Gastrointestinal diverticulae. Psychological The main problem with having Ehlers-Danlos syndrome is that the person can look very fit and may often not be believed that they have joint pain and other symptoms. Diagnosis is often delayed and misdiagnosis is relatively common. Some forms of EDS may be misdiagnosed as child abuse/self-inflicted injury. Where there is severe skin involvement, scarring can be severe and the person needs to learn to cope with disfigurement. Treatment This depends on the presenting symptoms but simple precautionary measures will greatly lessen the chances of accidental trauma, scarring and bruising. It is important to carefully balance the advantages of less frequent injuries and the disadvantages of over-protection in a child. Simple measures like padding of the lower legs and elbows in children may reduce the number of injuries. Surgery and stitching of skin injuries should be undertaken with great care as fragile tissues may tear. Stitches need to be left in longer than normal. Bracing and splinting may be used to support unstable joints. Orthopaedic surgery may be necessary but is not always successful. Physiotherapy and occupational therapy advice may be sought in order to strengthen muscles and to aid daily living. The prognosis depends on the specific type of EDS. Life expectancy can be shortened in the Vascular Type (type lV) due to the rupture of vessels and organs. Pregnancy can be life threatening in the Classical and Vascular Types (types l,ll,lV). For further information contact: Ehlers-Danlos Support Group PO Box 335, Farnham, GU10 1XJ. Reg. Charity No. 1014641. Tel/Fax: 01252 690940. E-mail: director@ehlers-danlos.org Website: http://www.ehlers-danlos.org/ Return to the Top of Page EPIDERMOLYSIS BULLOSA Epidermolysis bullosa is the name given to a group of genetically determined disorders, which are characterised by an excessive susceptibility of the skin and mucosae to separate from the underlying tissues following mechanical trauma. The individual diseases vary in their impact from relatively minor disability (e.g. limitation of walking distance because of blistering of the feet), to death in infancy. There are three broad categories of epidermolysis bullosa: epidermolysis bullosa simplex, dystrophic epidermolysis bullosa and junctional epidermolysis bullosa. Within each of these categories, there are several sub-types which are clinically, and probably genetically, distinct. Types and Causes EB Simplex - A group of inherited disorders characterised by mechanically induced blistering occurring within the epidermis itself, as a result of lysis of the basal keratinocytes (breakdown of epidermal skin cells). There are several established variants, of which the following are the most important: Weber-Cockayne type -epidermolysis bullosa localised to the hands and feet. Koebner type - generalised epidermolysis bullosa simplex. Dowling-Meara type - epidermolysis bullosa simplex herpetiformis. EB Simplex with muscular dystrophy. In addition, there are a number of rarer variants which are encountered from time to time. The prevalence of the different forms of epidermolysis bullosa simplex can only be estimated. Weber-Cockayne type is probably 10-20 cases per million population, Koebner is rarer, possibly about 2 cases per million and Dowling-Meara appears to be in the region of 5-10 cases per million. Almost all forms of epidermolysis bullosa simplex are inherited as autosomal dominant traits, although some rare forms are inherited as autosomal recessive traits. People with the generalised and the localised forms almost always have extensive family histories of the condition. Dystrophic epidermolysis bullosa - A group of inherited disorders characterised by mechanically induced blistering occurring immediately below the lamina densa of the basement membrane zone. These disorders derive the name dystrophic from the tendency of the blisters to heal with atrophic scarring. Dystrophic epidermolysis bullosa may be inherited as an autosomal dominant or an autosomal recessive trait. In general, it tends to be most severe when inherited as a recessive, and mildest when inherited as a dominant, but there is considerable clinical overlap. There are few data to indicate the prevalence of dystrophic epidermolysis bullosa. A recent study in Scotland indicated an estimated prevalence of 21.4 cases per million. Junctional epidermolysis bullosa - A group of inherited disorders characterised by mechanically induced blistering occurring within the basement membrane at the level of the lamina lucida. There are three broad types of junctional epidermolysis bullosa: Herlitz junctional epidermolysis bullosa - in which death is probable within the first two years of life. Non-Herlitz junctional epidermolysis bullosa - in which many people will survive into adult life. Junctional epidermolysis bullosa -with pyloric atresia. Because most affected individuals die early in life, the incidence of junctional epidermolysis bullosa is particularly difficult to ascertain. It is estimated that the incidence of new cases is approximately the same as for dystrophic epidermolysis bullosa, i.e. around 20 per million births. To date, all types of junctional epidermolysis bullosa have been transmitted as autosomal recessive traits. Symptoms and Treatments Due to the large number of different forms of epidermolysis bullosa with greatly differing symptoms and prognoses, it is unwise to try to describe these in any detail here. Broadly, the common features of the condition relate to blistering of the skin and mucosae at the slightest friction. In some forms the blistering is confined to particular areas of the body, such as the hands and feet. In others the blistering can affect all of the body. The blistered areas in some forms of epidermolysis bullosa heal normally, in others healing is accompanied by scarring. In view of this, specific information should always be obtained from a competent clinician who will be able to relate the information given to the precise form of epidermolysis bullosa that the person has. The patient support group DEBRA can provide the names of clinicians and other health care professionals who have a specialist interest in, and knowledge of, the condition. For further information contact: DEBRA, DEBRA House, 13 Wellington Business Park, Dukes Ride, Crowthorne, Berkshire, RG45 6LS. Reg. Charity No. 1084958. Tel: 01344 771961. Fax: 01344 762661. Email: debra.uk@btinternet.com Website: http://www.debra.org.uk DEBRA is an international not-for-profit organisation co-ordinating efforts to help change the lives of people affected by EB. DEBRA’s main activities are: funding medical and scientific research into EB. providing specialist advice by a team of nurses and social workers to people with the condition. DEBRA produces a range of information materials for people whose lives are affected by EB, and a range of materials for health professionals. DEBRA runs holiday homes in Brittany, and in Blackpool and Weymouth for people with the condition. Return to the Top of Page HERPES VIRUSES Herpes simplex causes cold sores, whitlows on the fingers/hands and genital herpes. By adulthood, herpes simplex infection in the UK is around 70%, worldwide it nears 100%. Herpes simplex can be differentiated into two genotypes, type 1 being much more common than type 2. Infection rates are detected by antibody tests since the majority of people infected are unaware of it, being asymptomatic. Recurrences only affect a minority of those infected and occur due to a lowered immune response which may be caused by illness, stress, tiredness, etc. There are nine known human herpes viruses. Herpes simplex and herpes varicella-zoster (chickenpox) cause skin conditions. The other viruses in the herpes family may cause malaise but do not affect the skin. Cause Herpes simplex virus types 1 and 2 produce sores: cold sores on the face, herpetic whitlows on the hands and fingers and “herpes” elsewhere on the body, especially on the genitals. The virus is transmitted by direct skin-to- skin contact with the affected part. The incubation period is usually 2-14 days, however asymptomatic carriers may develop symptoms years after first contracting it. Symptoms The primary illness last about 2-3 weeks and is self-limiting: flu-like symptoms followed by blisters (on ordinary skin) which open into sores or ulcers before scabbing over and healing. After the primary infection, herpes simplex virus remains latent in the body and may reactivate at a later date. If herpes simplex recurs, it will only affect the dermatome (nerve region) where infection took place causing one or more spots. These may be preceded by flu-like symptoms. Neuralgia, described variously as scalded skin, deep aches, tingling or shooting nerve pains, may be experienced in the dermatome either before or between outbreaks. Symptoms are not spread to other nerve regions. With time recurrences usually diminish both in frequency and Treatments If the primary infection of herpes simplex is severe, a course of antiviral drugs may be prescribed: aciclovir, Famvir or Valtrex. Otherwise all that is necessary is to alleviate symptoms using antipyretics, analgesics and topical anaesthetics as appropriate. Eye infection: Varicella-zoster virus may affect the ophthalmic division of the trigeminal nerve causing pain in, usually, one eye. Diagnosis is by fluoroscein stain. Treatment with antiviral ophthalmic cream as well as antiviral pills is recommended. Eye infection may damage sight or even, rarely, lead to blindness. If herpes simplex recurs with a frequency that the patient finds distressing, antiviral treatment may be given prophylactically for a period of six months (minimum useful treatment). Self-help is also often effective. The Herpes Viruses Association can offer suggestions, as well as counselling, so that the patient’s anxiety is ameliorated. Psychological Effects A diagnosis of herpes simplex can be very distressing. Since the early 1980s, inaccurate and deliberately misleading information has appeared in newspapers and magazines. Women, in particular, should be reassured in advance by the diagnosing doctor about two myths which they may come across: herpes simplex is not a causal factor for cervical cancer and women who catch herpes simplex prior to conception need not assume they will need a Caesarian section. A diagnosis of herpes simplex can often cause greater psychological misery than physical suffering. Sexually transmitted conditions carry a stigma, exacerbated in this country by an immature national attitude to sex, characterised by prurience and prudery. The ‘herpes hype’ and stigma associated with the condition means that most people remain secretive about the condition. For further information contact: Herpes Viruses Association (SPHERE) 41 North Road London N7 9DP Reg. Charity No. 291657 Tel: 020 7607 9661 (office and Minicom V) Tel: 020 7609 9061 (helpline – 24 hours access) Fax: on request Website: http://www.herpes.org.uk The Herpes Viruses Association and its officially recognised subgroup the Shingles Support Society (see “shingles and chickenpox”) have as their aims: to promote good health by advancing information about herpes viruses and the means by which these conditions may be most effectively prevented and treated to the public, media and medical professionals. to supply specific answers for the public and members of the association who are affected by the viruses through the confidential helpline run by trained volunteers who all have herpes simplex, letters or counselling. to provide members with leaflets on every aspect of “living with herpes”, leaflets, a lively and informative quarterly journal as well as workshops, seminars, local contacts and social events. “I thought my life was at an end and I would never have sex again,” wrote Darren. He is now in a relationship. Return to the Top of Page HIDRADENITIS SUPPURATIVA Hidradentis suppurativa is a disease of the apocrine sweat glands. It was first diagnosed as a condition by Velpeau in 1839 and in 1854 was associated with a peculiar inflammation of the sweat glands by Verneuil, who also named the disease. Hidradenitis suppurativa is sometimes referred to as: apocrinitis, apocrine acne, Verneuil's disease, Velpeaus disease, Fox-den disease, acne inversa or hidradenitis axillaris. Although not a fatal disease the ramifications, both physical and emotional, that accompany hidradenitis suppurativa, can prove difficult to overcome for people struggling with the condition. It is estimated to affect up to 3% of the world's population. Causes There are many theories about the causal factors for this condition, however a definitive cause remains elusive. Symptoms The disease affects the areas where the apocrine glands occur. During the first few weeks of foetal development these glands cover the entire body, but have receded and stabilised by the time of birth into the pubic regions. Here they stay dormant until puberty. The onset of hidradenitis is found most commonly between the ages of 11 and 30, although cases have been documented in children as young as 2 years. Tender red nodules develop in the apocrine glands of the axillae (commonly in women), in the groin and on the buttocks (commonly in men), which are firm at first, but later become fluctuant, painful and eventually rupture discharging pus. Hidradenitis develops in three stages and due to its insidious nature is normally misdiagnosed in its first stage as a single abscess or boil and is thus treated as such. It is only when the disease has reached stage II that the majority of accurate diagnoses are made. The spread of the condition is by a mechanism known as sinus tracting. This is caused by the effect of bacterial infection, and pressure from the resulting abscesses, forcing a pathway under the skin surface and infecting other tissues of the body. Types Stage I - Single or multiple abscess like growths in isolated incidences, with no scarring or sinus tract involvement. The possibility of long term remission at this stage is very high and people with the condition may have periods of up to several years between outbreaks. Stage II - Multiple or single abscess like growths in multiple regions, with possible sinus tract scarring beginning to occur. This stage is characterised by infrequent periods of remission. It is at this stage that the majority of diagnoses are made. Stage III - Multiple abscess like growths throughout the pubic areas; widely spread scarring from previous infections and formation of sinus tracts, giving rise to constantly weeping open wounds. Treatments Hidradenitis suppurativa is not a contagious disease and cannot be transmitted from person to person. There is no cure at present for the condition. However, if caught in the first stage, prompt surgical intervention can offer a good possibility of full remission. Some treatments have met with limited success and it is important to consider possible side effects of any course of treatment. For further information contact: H.S.S.Group UK & Ireland, 207 Fernhill Road, Bootle, Merseyside, L20 0AG. Website: http://www.hssg.org.uk Support Community website: http://www.communityzero.com/ ukhsgroup/ The Group aims to: provide support for people with hidradenitis suppurativa and their families. promote higher awareness of hidradenitis suppurativa within the UK and worldwide. educate doctors about the effects of the condition. have hidradenitis suppurativa recognized as a disability by the UK Government. establish funding for research into treatments and to find a cure for hidradenitis suppurativa. Return to the Top of Page HYPOMELANOSIS OF ITO Aneurocutaneous syndrome of streaky, patchy, whorl-like, or linear macular hypopigmentation of the skin, often associated with seizures, developmental and intellectual retardation, and other anomalies. Dr Ito first wrote in a Japanese medical journal in 1952 about some patients he had seen who had a pattern of skin markings in a V shape over the spine and in lines down the arms and legs. Over the years other doctors have written in medical journals describing one or two patients with the same skin markings. Some of the patients have had problems with development. Until recently no one knew the cause of Ito’s disease but the results of research carried out in Manchester have been confirmed by other studies in the USA. Differential Diagnosis: Ectodermal Dysplasias, especially Naegeli Type; Incontinentia Pigmenti (Bloch-Sulzberger Syndrome); Systematized Nevus Depigmentosus; Tuberous Sclerosis; carbohydrate-deficient glycoprotein deficiency Type III. Causes The body is made up of millions of cells usually each one has the same genetic instructions as each other (chromosomes and genes). In Ito’s disease there are two groups of cells, one with one set of genetic instructions and another with a slightly different set of instructions. It is the upset in “balance” between the two sets, which causes the problem. In the type with the extra chromosomes, usually the egg and the sperm are normal when they join together. The fertilised egg then divides and it is at this division or one of the following divisions that the chromosomes do not divide equally giving rise to a set of cells with an extra chromosome. In the type with the gene variation the egg and sperm contain normal gene instructions and when the fertilised egg divides a gene fault occurs in one of the cells and thereafter all the cells resulting from that cell contain the faulty gene. The above problems just happen by chance and are not caused by anything the parents have done and are not usually inherited. Symptoms These include asymmetry of the body, patchy pigmentation which can occur on any part of the body, but not normally the palms, scalp or soles of the feet. Gross motor and psychomotor retardation, epilepsy, scoliosis, dental and bone anomalies, unusual ophthalmic features and kidney problems, amongst others. How a child is affected depends partly on the particular chromosome or gene fault and partly on how many cells contain the fault. The sort of problems children with Ito’s disease have can be divided into two types: Physical and Developmental. Not all children have all of the problems. Physical problems These include problems obvious at birth such as unusual shape of joints, extra fingers, etc. Many children with Ito’s disease have more growth on one side of the body than the other making them a little asymmetrical. The skin patterns are usually first noted when a child is a few months old. Developmental problems Some children with Ito’s disease make very good progress and others are slow with their development. There is no way other than careful follow-up to predict how a particular child will develop. Even those children with problems with development do continue to grow and make progress although slower than other children. Extra help in nursery and school may be necessary. Treatments Since there is no cure for Ito’s disease, treatment has to be symptomatic. Epilepsy is treated with drugs (or occasionally surgery); Behavioural difficulties (including hyperactivity and autistic features) are often the most troublesome for families and frequently require appropriate intervention. Scoliosis (curvature of the spine) can be helped by the wearing of a brace and in some cases surgery. There is no treatment for the depigmentation markings of the skin For further information contact: HITS (UK) Family Support Network (supporting families affected by the Hypomelanosis of Ito Syndrome) National Contact: Sandra Field 33 Fernworthy Close, Shiphay, Torquay, Devon TQ2 7JQ Tel: 01803 401018 London Contact: Terri Grant 07940 114943 Helpline Tuesday evenings 7-9 p.m. only (UK) time 020 8352 1824 Email: tgrant@uk.ey.com or tgrant@hitsuk.freeserve.co.uk Webpage www.e-fervour.com/hits HITS (UK) is a family support network which was specifically set up by families to support other families with this syndrome because no other support group existed anywhere else in the world. HITS (UK) aims to support families by letter, e-mail, telephone, internet chat room, 3 newsletters a year and by bringing families together at events to reduce the sense of isolation often felt. The Family Support Network’s aim is: To enrich the lives of families and children affected by Hypomelanosis of Ito by facilitating and encouraging communication and linking families together, and by being a focal point offering verbal and written support : To put families who have a common situation or difficulty in touch with each other to enable them to gain mutual support and friendship. To reduce the sense of isolation families and children with disabilities or special needs often feel. To liaise with the medical profession to communicate the group’s existence and to share medical information available to us. To have quarterly conference calls and to produce a quarterly Newsletter. To organise an Annual or Bi-Annual Family Event. To promote the importance of the group and the need for communication. To organise medical training awareness days in an attempt to gain earlier diagnosis. Ignorance creates disillusion – Awareness ends confusion Return to the Top of Page ICHTHYOSIS Ichthyosis is a term used to describe continual scaling of the skin. It comes from a Greek word ‘ichthys’ which means fish. It is quite rare and can be inherited (genetic or congenital) or it can develop later in life (acquired). The inherited forms of ichthyosis are usually evident at, or soon after, birth and they tend to persist throughout life, although some types improve with age. Most, if not all, of the skin is affected. Ichthyosis can cause severe psychological problems. The public’s reaction to its appearance is often hostile and unsympathetic. Staring and teasing are common. Causes Faulty copies of genes, which are passed on from one generation to the next, cause inherited ichthyosis. Each type of ichthyosis is due to a different genetic mutation and the pattern of inheritance varies. Recent research on bullous ichthyosis has led to the discovery of a particular mutation to the gene that controls the skin protein keratin. Other types of ichthyosis have been shown to be related to abnormalities in the lipid or fat chemicals in the skin such as in X-linked ichthyosis. Symptoms These can vary greatly between the different types of ichthyosis (see individual types). Darkened and rough skin can appear as scales separated by deep creases. In some cases skin blisters and peels. The main forms of inherited ichthyosis are as follows: Ichthyosis vulgaris - The most common form and usually quite mild, with fine scaling or roughness on the arms and legs. X-linked recessive ichthyosis - Occurs in men and boys and causes brownish flat scales mostly to the arms, legs, and tummy. Varies in severity and some affected babies have a difficult delivery. Genetically, it is passed on through the female line. Non-bullous ichthyosiform erythroderma and lamellar ichthyosis - These are very rare, are often severe, with prominent scaling, and cause redness over most of the skin. Affected babies are often born with a shiny waxy second skin (collodian membrane), which sheds in a few days. Bullous ichthyosis - This is another rare red ichthyosis, which causes blistering, fragile skin early in infancy and thick scaling especially around the joints later on. Harlequin ichthyosis - This condition is an extremely rare but very severe form of ichthyosis, which causes thick plates of scale and severe complications at birth. Many affected babies do not survive. Netherton’s syndrome - The affected infant has red inflamed scaly skin from birth, fragile spiky hair and difficulty gaining weight in early childhood. Sjogren–Larsson syndrome - Another condition that also produces scaling and thickening of the skin which may not be obvious until a few months of age. Affected babies also have a type of cerebral palsy. Conradi-Hunermann syndrome, neutral lipid storage disease and KID syndrome - These are very rare and are associated with other medical problems. Treatments The milder ichthyoses can be well controlled using regular moisturisers (emollients), including bath oils, soap substitutes and moisturising creams. The more severe forms may make affected babies quite unwell and treatment in special care baby units will give them a good start. Moisturisers are again important as an ongoing treatment and antibiotics may be needed from time to time. A group of drugs called retinoids may be tried as they lessen the scaling in many forms of ichthyosis. The complicated varieties of ichthyosis will need additional treatments according to their severity. As yet there is no cure for ichthyosis. For further information contact: Ichthyosis Support Group (ISG) People with ichthyosis and carers: ISG National Contact, 16 Cambridge Court, Cambridge Avenue, Kilburn, London, NW6 5AB. Tel/Fax: 020 7461 9034 (after 8pm). Medical professionals: ISG Secretary, 2 Copnor, Woolton Hill, Nr. Newbury, Berkshire, RG20 9XH. Tel: 01635 253829. E-mail: isg@ichthyosis.co.uk Website: www.ichthyosis.org.uk The aims of the Ichthyosis Support Group are to: provide support, encouragement and information on ichthyosis. raise awareness of ichthyosis, not only amongst the medical profession but also to the public. promote research into treatments for ichthyosis. The Ichthyosis Support Group provides: a quarterly newsletter. an information pack with literature on various forms of ichthyosis and skin care tips. national get-togethers. a base of interested medical professionals. a pen pal scheme. Return to the Top of Page LEG ULCERS & PRESSURE ULCERS Leg ulceration is a chronic disease occurring predominantly in the older population. An ulcer can be defined as a local deficit or excavation of the surface of an organ or tissue, which is produced by the sloughing of inflammatory necrotic tissue. Ulcers may be present for many years but can be successfully treated following a comprehensive medical assessment, appropriate clinical management and patient compliance to treatment. Estimates of prevalence range from 1.5 to 1.8 per 1000 total population, rising to 3 per 1000 at age 61-70 and to 20 per 1000 in people aged 80 and over. The annual cost to the National Health Service (NHS) is estimated to be in the region of £300-600 million. The majority of patients are cared for bycommunity services. Venous ulcers account for 70-90% of all cases. Arterial ulcers account for 5- 20% of cases. Combined venous hypertension and arterial disease account for 10-15% of cases. Less common causes account for 5-10% of cases and include: diabetes, vasculitis, neoplasm, infection and trauma and other less specific causes. Causes and Symptoms Venous ulceration can result from chronic venous hypertension in the lower limb, usually due to malfunctioning valves in the leg veins, of either congenital or acquired origin, resulting in a backflow of blood. The superficial venous network is exposed to higher pressures than normal resulting in oedema, capillary damage and thinning of the dermis (inner layer of the skin containing vessels, nerves and fibrous tissue - covered by the epidermis). The subsequent leakage of red blood cells and large protein molecules creates the physiological conditions which can lead to ulcer formation. Varicosities (abnormal swelling of veins) are commonly present. Often the lower limb is swollen with a brown discoloration of the skin and the presence of an irritating eczema. Arterial ulcers result from a reduced arterial circulation. Common causes include atherosclerotic changes in the main vessels and small emboli (blockages), not uncommonly the result of hypertension. Smoking and poor diet are contributory factors. The lower limb may appear pale or a dusky red colour and the patient commonly complains of pain, particularly when the limb is elevated or during exercise. Leg ulceration impacts on the quality of daily life through pain, physical restrictions, sleep disturbance, reduced energy, emotional reactions and social isolation. Treatments Venous ulceration - treatment aims to counteract the high pressure in the superficial veins, increase the calf muscle pump mechanism to assist venous return and reduce oedema. This is achieved by applying graduated compression from the toes to the knee. Local wound management aims to achieve optimum healing conditions and avoid the exacerbation of varicose eczema. Underlying nutritional deficiencies should be corrected. The prognosis is good, providing the cause of the ulcer has been correctly assessed and management decisions have involved patient consultation. Support or compression hosiery is often necessary to prevent recurrence following healing. Arterial ulceration - treatment includes the local management of the wound and avoids compression, which would exacerbate the arterial insufficiency. Vasodilator drugs may be used. The prognosis is poor, unless surgical intervention is possible to correct the arterial problems. Cessation of smoking, weight control, a nutritious diet and maintaining mobility are beneficial. PRESSURE ULCERS A pressure ulcer is an area of damaged skin and tissue caused by unrelieved pressure, shear or friction, typically sustained during long periods of bed or chair rest during illness or disability. Prevalence rates are estimated at between 7% and 10% in hospital and community patients. It is suggested to be costing the NHS in excess of £400 million per year. Treatment is aimed towards prevention, including relief of the pressure and avoidance of friction and shear forces. There are many pressure relieving aids available and methods of care aimed at prevention. Damaged skin should be covered with a suitable dressing. For further information contact: Tissue Viability Society, Glanville Centre, Salisbury District Hospital, Salisbury, SP2 8BJ. Reg. Charity No. 1041915. Tel: 01722 429057. Fax: 01722 425253. Email: tvs@dial.pipex.com Website: www.tvs.org.uk The Tissue Viability Society is concerned with the development of good practice in the management of wounds of all types, particularly chronic wounds such as pressure ulcers and leg ulcers. The society does so by: maintaining a multi-disciplinary network of health care professionals to share and disseminate information. providing education through conferences and study days. publishing a quarterly Journal of Tissue Viability. providing a range of information booklets to the public and professionals. Wound Care Society, PO Box 170, Hartford, Huntingdon, PE29 1PL. Reg. Charity No. 1013304. Tel/fax: 01480 434401. Email: wound.care.society@talk21.com Website:http://www.woundcaresociety.org Return to the Top of Page LICHEN SCLEROSUS Lichen sclerosus is a chronic inflammatory skin condition resulting in well-defined shiny white spots on the skin. The condition can be seen in all ages but occurs more frequently in women of middle age. The condition more commonly affects women than men. Symptoms Lichen sclerosus most commonly affects the genital area. The predominant symptom is acute (intractable) itching of the vulval area. There may also be soreness and pain. Blood blisters and sores can occur, especially where the skin is scratched. The vulva thickens and looks white. Scarring may occur. Splitting of the skin can occur in the vulval and perianal area, especially on passing bowel movements. Atrophy and change in the vulval features occur in long established cases and include fusion of the labia over the urethra, causing difficulty in urination. The clitoris may become ‘buried’. There may be narrowing of the vaginal opening making sexual intercourse difficult. Infections (including ‘thrush’) may be present. In men the penis is affected by itching, soreness, difficulty in retracting the foreskin and, due to a narrowing of the urethra, in urinating. The skin may look pale. There is a small risk of cancer currently estimated at around 3%. The condition can also affect other areas of the body, for example the shoulders. Children are similarly affected. Psychological One of the main problems is recognition and appropriate diagnosis by the General Practitioner. Misdiagnose of the condition as ‘thrush’ is relatively common, or as a psychological complaint to be treated with anti-depressants and tranquillisers. An accurate diagnosis is delayed for many years in some people. Earlier specialist referral would reduce the distress caused to people who fail to respond to treatments for infections. There is a tendency for those experiencing painful sex to have a sense of low confidence and self worth, to feel they are freaks, to experience a loss of femininity/masculinity. Many grieve for their sexuality. There is often a very real feeling of total isolation and despair. Patients have difficulty in coming to terms with the disabling effect lichen sclerosus can have on everyday life. Treatments There is, as yet, no cure for lichen sclerosus. Treatments aim to relieve the symptoms of the condition by the use of topical steroid creams. Surgery is not part of the treatment but is sometimes necessary to separate fused labia and to relieve the symptoms of scarring. For further information contact: National Lichen Sclerosus Support Group, PO Box 7600 Hungerford Berkshire, RG17 7XD (enclose SAE for reply). Email: fabia.ivy@pop3.hiway.co.uk Website: www.lichensclerosus.org The National Lichen Sclerosus Support Group: offers support on a one-to-one basis, subject to demand information where current and available. endeavours to raise awareness and to educate medical professionals on the needs of patients with the condition. produces information leaflets. Return to the Top of Page LUPUS Lupus is an autoimmune disease, a type of self-allergy whereby the patient's immune system overproduces antibodies which then attack the person's own tissues. Lupus is neither infectious nor contagious, and its effect is inflammatory. Patients principally suffer extreme fatigue and joint and muscle pains. A variety of other symptoms are possible, as well as damage to vital organs if the illness has taken a strong hold. Nine out of ten people with lupus are female, and whilst it is usually triggered between the ages of 15 and 50, children can also have the illness. Some 50,000 people in the UK are thought to have lupus, many yet to be diagnosed, with the incidence of the disease being greater in Afro-Caribbean and Asian communities. Causes The causes of lupus are not fully understood, although heredity, puberty, hormonal activity and change, childbirth, viral infections, sunlight, the menopause, trauma or strong medication may play a part in triggering the illness. Lupus can affect people of all ages and both sexes. The predominance of the illness seen in females is due to the influence of hormonal factors. Symptoms Lupus can present in a complex number of ways, even to the extent of 'mimicking' other illnesses such as rheumatoid arthritis, multiple sclerosis (MS) and myalgic encephalomyelitis (ME). Lupus can be hard to diagnose and the condition can be overlooked, sometimes for years, unless the physician is alert to its possibility. Many patients differ in symptom patterns, which are taken into account with specific blood tests prior to diagnosis. A skin problem does not necessarily occur in systemic lupus (SLE) but is always present in discoid lupus (DLE), where rashes occurring on the face, scalp or neck can be quite severe. Both types arise from a disorder of the immune system. DLE can develop into systemic lupus, but does not always do so. It is most unusual for the SLE patient to develop DLE. In summary: SLE - acute, subacute or chronic skin rashes may occur, mainly on the arms and upper body, together with the possibility of a 'butterfly' rash on the cheeks. DLE - disc-shaped lesions can occur on the face, neck or scalp. Lupus is a weighty burden for the patient, being incurable and causing physical limitations and pain. Continuous resolve is needed by patients and their families to ensure that they are not 'dragged down' by lupus and are able to restore and maintain some quality to their lives. Treatments Both SLE and DLE skin conditions respond to treatment, but may recur where the underlying illness flares from time to time. The reasons for flare-ups are not always well understood. There is, as yet, no cure. Careful monitoring of the illness together with a flexible treatment programme, enables the condition to be controlled in the majority of patients. Treatments may range from no medication at all in very mild lupus, through NSAIDs (non-steroidal anti-inflammatory drugs) and corticosteroids to immunosuppressives and even stronger medication, where the illness is more serious. As the patient's lupus becomes better managed, the physician will try to reduce the volumes and/or levels of medication, eventually to reach the lowest possible maintenance level. Patients can learn to reduce the impact of the illness by becoming better educated about the condition, by learning to 'pace' their daily routines, by taking regular rest and by reducing the incidence of stress, depression, anger and pain wherever possible. For many lupus patients, lifelong medication and care is needed, yet in a smaller percentage the illness has been known to recede, particularly after the menopause. Physicians now have much greater knowledge of this still-mysterious disease and have a wider range of drugs at their disposal, which have made for easier control of the individual’s own ‘brand’ of lupus. Good information is also available to patients through lupus consultants, specialist nurses and support groups. For further information contact: LUPUS UK, St James House, Eastern Road, Romford, Essex, RMI 3NH. Reg. Charity No. 1051610. Tel: 01708 731251 (5 lines) and 24 hour Answerphone. Fax: 01708 731252. Email: headoffice@lupus-uk.freeserve.co.uk Website: www.lupusuk.com LUPUS UK works to support people with incurable lupus and their families by: making available information on lupus, its diagnosis, treatment and management. providing help through the charity's Regional Group and National Contact structure. promoting public awareness of lupus and information for the medical profession. maximising income for essential research into the causes and towards a cure. Return to the Top of Page LYMPHOEDEMA Lymphoedema creates distressing, debilitating and uncomfortable swelling. Lymphoedema most commonly affects a limb but the face, neck, abdomen, genitals and internal organs can be involved. It is still not a well-documented condition, but early diagnosis and treatment are essential for the optimum management of the disorder. Symptoms, Types and Causes Lymphoedema is a serious disorder for which there is no cure and which, without the appropriate treatment can gradually deteriorate. A worsening of the condition is not limited to just the increase in size of the limb but also to the insidious changes in tissue structure, e.g. elephantiasis, and susceptibility to frequent acute inflammatory episodes (infections) with the resultant long-term dependency on antibiotics. The physical and psychological impact on the patient with a swollen limb is immense. Extreme discomfort, heaviness in the affected part and problems of movement can be experienced. Over time, there may be a gradual thickening of the skin at the affected site and spontaneous lymphorrhea can occur. Repeated acute inflammatory episodes sometimes resulting in hospitalisation are not unknown. The quality of life is greatly diminished. Clothes and shoes that fit are difficult to find. The person has to adapt to an altered and more limited daily lifestyle. There is reduced working capability, intolerance to heat (climatic or in a sauna or baths for example) and minimal participation in social activities. Primary lymphoedema - is due to a fault within the lymph conducting pathways, i.e. no identifiable cause. It is usually determined from birth and may be due to malformation of the lymphatic system. Swelling develops progressively at different stages in life and presents usually in the lower limbs. It is normally indolent but associated discomfort and debility are very common. Congenital lymphoedema can also be associated with numerous developmental abnormalities. There are several different types of primary lymphoedema: Nonne-Milroy disease - congenital familial lymphoedema presenting at birth or soon thereafter. Any part of the body may be affected. Lymphoedema Praecox - onset is usually during puberty and represents some 80% of all primary lymphoedemas, usually affecting the lower limbs. Lymphoedema Tardum - onset is after 35 years of age. Secondary lymphoedema - is when the cause of lymphatic system failure is known, e.g. surgery, radiotherapy, infection, trauma such as severe injury, burns or perhaps thrombosis. The progression of lymphoedema can be classified in three stages: the reversible stage when swelling is soft and will pit easily with light finger pressure. The lymphoedema will disappear with bed rest. the spontaneous irreversible stage when the swelling hardens, pitting is not so obvious and there is the slow formation of fibrous tissues (fibrosis) where protein-rich fluid has collected. the elephantiasis stage when there is extreme swelling and thickening of the skin. Treatments Drug therapy is of limited value but diuretics are still recommended to many patients. These, in fact, have little or no effect on lymphoedema and can cause long-term problems. Pressure pumps are contra-indicated except under strict medical control. Surgery, such as 'debulking' or 'reduction' is a procedure of last resort. A person with lymphoedema, or at risk from lymphoedema following surgery, should never allow blood to be drawn, blood pressure to be taken or injections given in the affected limb or body part as these procedures may well manifest or exacerbate the condition. Lymphoedema cannot be cured but it can be controlled with early diagnosis, correct and early treatment and ongoing management. Decongestive Lymphatic Therapy (DLT) otherwise known as Complex Decongestive Therapy (CDT) has been the recommended treatment for lymphoedema for many years. Initially, an intensive course of treatment can last from ten days to six weeks. Following a course of treatment, compression garments will usually have to be worn indefinitely to maintain the volume reduction and tissue improvement achieved. It is also necessary to ensure that the body weight remains, or is brought within, normal limits. After six months to a year, a repeated course of intensive treatment may be necessary. In the meantime, daily exercises and, in an ideal world, regular manual lymphatic drainage therapy should be recommended. Patient compliance has to be 100% in following the medical recommendations on a daily basis between treatments and often the patient will have to reconsider their work and social lifestyles in order to comply. The overall benefit is that the lymphoedematous region is returned to as near normal as possible. The patient can enjoy an improvement in their quality of life and there are fewer, if any, episodes of cellulitis or other life-threatening infections. For further information contact: Lymphoedema Support Network, St Luke's Crypt, Sydney Street, London, SW3 6NH. Reg. Charity No. 1018749. Tel: 020 7351 4480 (answerphone out of hours). Fax: 020 7349 9809. Email: ADMINLSN@lymphoedema. freeserve.co.uk Website: http://www.lymphoedema.org/lsn Available to members: quarterly newsletter - LymphLine. up-to-date national/international information on lymphoedema treatment. talks from medical experts. bi-monthly group meetings at the Royal Marsden Hospital, Chelsea. general leaflets and reading list. For professional enquiries only, contact: British Lymphology Society, 1 Webb’s Court, Buckhurst Avenue, Sevenoaks, Kent, TN13 1LZ Reg. Charity No. 1042561. Tel: 01732 740850 (answerphone out of hours). Fax: 01732 459225. Website: www.lymphoedema.org/bls Email: admin@blsac.demon.co.uk Return to the Top of Page NEUROFIBROMATOSIS Neurofibromatosis is a genetic disorder of the nerve tissue presenting in two forms. Type 1 (Nf1) affects one in every 2,500 people world-wide. This equates to more than 23,000 people in the UK. An affected person of either sex has a 1-in-2 chance of passing the defective gene on to any, or all, of their children. However, everyone is at risk because 50% of cases result from spontaneous mutation in families with no previous history of neurofibromatosis. Type 2 (Nf2) affects one in every 35,000 people world-wide (more than 1,500 in the UK) and, as with Nf1, an affected person has a 1-in-2 chance of passing on the defective gene to any of their children. Symptoms Type 1 (Nf1) - Six or more coffee coloured (cafe-au-lait) marks on the skin in the first five years of life and/or nodules (lumps and bumps) on or just below the surface of the skin. Complications include: learning difficulties behavioural problems high blood pressure (hypertension) curvature of the spine (scoliosis) malformation of the long bones (below the knee and below the elbow - pseudarthrosis) large benign skin tumours (plexiform neurofibroma) tumours on the nerves of sight (optic glioma) internal, spinal and brain tumours -usually benign speech problems increased risk of epilepsy hearing defects can lead to paralysis can cause premature death Type 2 (Nf2) - The chief characteristic of Nf2 are bi-lateral acoustic neuromas (tumours on both nerves of hearing). Unless there is a family history of Nf2, diagnosis is often very difficult. With no outward signs, in most instances substantial hearing loss has occurred before Nf2 is suspected. Surgery is often the only treatment and facial paralysis is, in many cases, unavoidable. Nf2 is always serious. Complications include: benign brain tumours (meningiomas) tumours of the spine (meningiomas, schwannomas) cataracts skin tumours (schwannomas) can lead to paralysis can cause premature death Treatments In every case, prognosis of Nf1 is uncertain; no two cases are the same. One of the particular problems is that the course of the disease cannot be predicted even within families; the disease complications do not breed true. The morbidity and mortality of the disease is largely determined by which complications develop. Recent studies, however, have drawn attention to the significant morbidity in terms of psychological well-being caused by the cutaneous neurofibromas themselves. There is no actual preventative treatment for any of the disease features. Treatment for many of the complications is the same as when they occur in isolation in the general population. With regard to neurofibroma removal, there seems to be no particular benefit derived from either surgical or laser treatment. Nf2 is consistently a more severe problem than Nf1. Nearly all affected individuals eventually develop bilateral acoustic neuromas which are very difficult to treat successfully. There is no useful medical treatment for the tumours at the moment, all of which need to be removed surgically. Patients need to be referred to centres with established expertise in acoustic neuroma surgery. For further information contact: Neurofibromatosis Association, 82 London Road, Kingston on Thames, Surrey, KT2 6PX. Reg. Charity No. 1078790. Tel/Answerphone: 020 8547 1636. Fax: 020 8974 5601. Minicom: 020 8392 0184. Times: Monday-Friday: 9:00am - 5:00pm Email: nfa@zetnet.co.uk Website: http://www.nfa-uk.org.uk The Neurofibromatosis Association is a national charity which has three main areas of work: building up a national network of Support Co-ordinators mainly based at Regional Genetic Centres; there are currently 12 plus one national Nf2 Co-ordinator. They can give real support to families affected with neurofibromatosis and their work can assist GPs in the management of cases of the disorder. providing information for patients, the public and medical and educational professionals. The Association is the only source of accessible information. It produces a quarterly newsletter, books, leaflets and videos. raising funds for research and, through a Medical Research Board, directing those funds to the best scientific advantage. Return to the Top of Page PEMPHIGUS VULGARIS Pemphigus vulgaris (PV) is one of a group of chronic, relapsing auto-immune diseases causing blistering of the skin and mucosal membranes. In pemphigus disorders the immune system perceives the skin and mucous membranes as foreign and an immune response is triggered. Although the condition is controllable with heavy immunosuppressive treatment there is no cure available and, if left untreated, the disease can prove fatal. Onset usually occurs in middle age (4th to 6th decades). There are no definitive statistics but worldwide incidence of all types of pemphigus is thought to be between 1 and 5 people per hundred thousand affected. PV is very rare, with most General Practitioners never encountering the disease, which can lead to mis-diagnosis of the condition. Causes Research studies show a genetic predisposition to the disease, but although researchers have identified a variety of possible triggers, these remain speculative. The possible triggers suggested, include: D-penicillamine derived drugs, some viruses e.g. Epstein-Barr and herpes simplex, some food groups particularly the allium family, exposure to the sun, environmental pollutants and long-term stress. Affected individuals have high concentrations of antibodies produced by the immune system. These bind to a specific protein in the skin (desmoglein 3), causing interference with the skin’s normal function and separation of epidermal cells. This often occurs first in the mouth, but lesions can cover a significant area of skin. The disease is non-infectious. Symptoms Someone with PV may feel ill a lot of the time, even if the disease is under control. A patient can be either in remission or have the condition under control, but still experience some blistering lesions. These burn-like lesions often first occur in the mouth and throat, are extremely painful, and are frequently mis-diagnosed as ulcers. The sensation has been compared to having a candle burning in the throat. Consequently pain control is a crucial issue. Lesions on the skin sometimes hurt, and even if they are not painful, they often itch and burn continuously, which in itself is physically upsetting for the patient. The pain endured by people with the condition can be very distressing, not only for the person concerned but also for carers. Some people manage the high levels of corticosteroid and immunosuppressive drug therapies without difficulty, whilst others find that, apart from long-term side-effects (eg. osteoporosis), the daily consequences of using such drugs produce health problems which are difficult to live with. Due to its rarity, people often find it hard to get accurate and up-to-date information on the condition and most people feel extremely isolated. Treatments The standard treatment is with corticosteroids, which are often started at high doses (eg. 60-100mg per day), together with immunosuppressive drugs (Azathioprine, Cyclophosphamide, Mycophenolate mofetil, Cyclosporin). Other drugs that are used with varying effects are: Methotrexate; Dapsone; Gold injections; Tetracycline, minocycline or doxycycline combined with niacinamide . Drug doses are reduced slowly to the minimum required to maintain the condition under control (which varies from individual to individual). Topical therapies are sometimes given in conjunction with systemic drugs to help heal recalcitrant erosions. In the pre-corticosteroid era, PV was generally fatal. Even with steroids and immunosuppressives, it remains a difficult disease to treat and has a mortality rate of about 6%. For further information contact: Pemphigus Vulgaris Network, Flat C, 26 St Germans Road, London, SE23 1RJ. As the group has no funding please could you enclose a stamped addressed envelope. The Pemphigus Vulgaris Network offers general support and: provides a forum for people living with PV and those personally or professionally concerned with it. gives people an opportunity to contact other people with PV. helps people find information they need. The Network also offers support to people living with Mucous Membrane Pemphigoid (including Ocular Pemphigoid). The PV Network is on the British Association of Dermatologists’ register of support groups and is affiliated to the International Pemphigus Foundation (The Atrium Plaza, Suite 210, 828 San Pablo Avenue, Albany, CA, 94706, USA. Tel: 001-510-527-4970. Fax: 001-510-527-8497. E-mail: pemphigus@pemphigus.org The Network shares a website with the International Pemphigus Foundation (http://www.pemphigus.org). Return to the Top of Page PSEUDOXANTHOMA ELASTICUM Pseudoxanthoma Elasticum (PXE) is an inherited disorder in which elastic fibres, which are normally found in the skin, the retina of the eyes and the cardiovascular system become slowly calcified, producing characteristic changes in these three areas. It is estimated that about 1 in 70,000 people in the world has PXE and for no known reason the condition affects twice as many females as males. The average age of onset is about 13 years with a range from 2 to 20 years. The cause of the disease is unknown. Symptoms The first manifestation, and the one which nearly always makes the diagnosis possible, is the appearance of highly characteristic, slightly thickened, patches of skin. The skin changes seen in PXE are variable. Some individuals experience very little change, whilst in others the small 2-5 mm yellowish or yellow-orange papules may form groups or coalesce into larger plaques giving a cobblestone or plucked chicken appearance. The skin may become lax or folded. The appearance and location of skin lesions can cause psychological distress. They most commonly first appear on the sides of the neck, which leads to younger persons being accused of not having washed properly. Lesions later appear in other flexural areas, for example under the arms, folds of the arms, the groin and behind the knees. The diagnosis can be confirmed by a skin biopsy, a procedure in which a small piece of skin is removed for analysis. In PXE, the biopsy will show a distinctive calcification of the elastic fibres. Widespread involvement of the body is rare. In some cases the onset of skin lesions may be delayed until late in life, but many such individuals probably had small skin lesions for many years which were unrecognised as PXE. In a few individuals, suspected of having PXE, the skin involvement is so mild it may be thought to be lacking, but very careful examination, or a skin biopsy, will confirm a diagnosis. Involvement of other systems: As has already been mentioned, the effects of PXE can go far beyond those affecting the skin, which are the most characteristic effects of PXE. The ocular and cardiovascular manifestations are responsible for the morbidity of the disease. The eyes of 60% of those with PXE will be affected eventually, usually in middle life. A smaller number will suffer from cardiovascular problems affecting the heart and legs (intermittent claudication), and from gastrointestinal bleeding. Treatments Skin lesions are asymptomatic, causing no medical problems in themselves, but can be cosmetically objectionable if they become severe. There is no specific treatment to change the appearance of the skin lesions and skin folds, other than plastic surgery. Good cosmetic results have been achieved on the neck, however plastic surgery on the axillae is more difficult. Some improvement is possible if the skin sags excessively in these areas. Attention to a proper, balanced diet and control of calcium intake will help slow down the process which affects the skin. Calcium is needed for life and a normal physiology, but not in excessive amounts. A calcium intake of no more than 800mg per day is recommended. If necessary a dietitian should be consulted to help maintain a balanced diet. Research The current research in all hereditary disorders is to look for the defective gene responsible for the condition and then attempt to add or replace the missing or altered substance. In May 2000, the good news that the PXE gene had been isolated was sent round the world. The gene has been identified as 'ABC-C6'. Researchers are now addressing the question of how and why a loss of function of the ABC-6 leads to PXE. The answer to this will hold important clues towards treating and/or preventing the illness. For further information contact: Pseudoxanthoma Elasticum Support Group (PiXiE), 15 Mead Close, Marlow, Bucks. SL7 1HR. Reg. Charity No. 1055465. Tel: 01628 476687. Fax: 01628 486024. Email: PXEeurope@aol.com Website: http://www.pxe.org.uk/ The Pseudoxanthoma Elasticum Support Group was founded to: help, encourage and assist those with PXE and their family members. heighten the awareness of PXE amongst the medical community, support services and the public. link members with each other to provide mutual support and for an exchange of ideas. provide information on the various aspects of PXE. provide copies of the newsletter PiXiE, containing medical articles and letters from members. hold annual meetings to which all members, their families and friends are invited. Return to the Top of Page PSORIASIS There are several forms of psoriasis, which usually appear as patches of silvery scales on top of areas of crimson skin. The scales are easily shed or scratched off. It is a distressing condition, which can lead to a reduced self-esteem. Over a million people in the United Kingdom and Ireland express the condition, equating to 2% of the population; with men and women being equally affected. Psoriasis usually occurs between the ages of 10 and 45 years, although there are exceptions. It is an inherited condition, which does not necessarily recur in successive generations. Causes Psoriasis is caused by the over production of skin cells. The development of skin cells to replace those naturally sloughed off occurs at up to seven times the normal replacement rate. Hence, raised red patches are produced. These can be covered with scaly, dead skin. Trigger factors for a psoriasis flare-up include: infection; damage to the skin (burns, sunburn and scratching for example) and, certain medications (eg. anti-malarial, anti-depressant and beta receptor blocking drugs). Very commonly, stressful events such as death, divorce, examinations and work pressures may precipitate a flare-up or may exacerbate a mild flare-up. In women, hormonal changes can affect the condition. The basic cause of the condition remains unknown and is subject to ongoing research. Types and Symptoms Chronic plaque psoriasis (psoriasis vulgaris) - This is the most common type, appearing on elbows and knees, or sometimes more extensively over the trunk and limbs. Pustular psoriasis (palmar plantar) - Pustules on the soles of the feet and palms of the hand, which go brown and develop scales. The skin often cracks. More often seen in middle age. Flexural psoriasis - This type appears in the armpits, groin and under the breasts. It is fiery, shiny red, with little or no scaling. Guttate psoriasis - This is quite common in children and teenagers, often occurring after a streptococcal throat infection. Lesions appear as small ‘raindrop’ patches. Psoriasis of the scalp and nails -Scalp psoriasis affects the majority of people who develop the condition. Scaling occurs, especially around the hairline. It is often itchy and can sometimes lead to a temporary loss of hair. Nail involvement is less common. Nails may show pitting, flaking and ridges. This can be an early indication of psoriatic arthritis (see section on Psoriatic Arthritis). The lowering of self-esteem is one of the most profound aspects of psoriasis. With such an emphasis on appearance in society, the incidence of psoriasis can cause a great deal of distress. One of the most difficult aspects is its effects on everyday life. A reduced self-esteem can affect the freedom to sunbathe or swim, visit the hairdresser or try on new clothes in a store and sometimes the choice of career. Having to cover up in warm weather and feeling unable to pursue the activities of people who are unaffected by the condition, add to the debilitating features of living with psoriasis. Temperature regulation is affected and people with the condition can feel hot when others feel cold, and vice versa. Sleep can be difficult. Itching and soreness can exacerbate the condition, leading to a general feeling of being unwell. It is important to recognise that psoriasis is a non-contagious condition and cannot be spread by touch to unaffected areas of skin or to other people. Treatments Mild to moderate psoriasis is treated with topical applications containing coal tar, dithranol, or the novel Vitamin A and D derivatives. Ultra violet light therapy and immunosuppressants are used in more resistant cases. For further information contact: Psoriasis Association, Milton House, 7 Milton Street, Northampton, NN2 7JG. Reg. Charity No. 257414. Tel: 01604 711129. Fax: 01604 792894. Email: mail@psoriasis.demon.co.uk Website: www.psoriasis-association.org.uk Psoriasis Association helps people with psoriasis and psoriatic arthritis by: collecting funds for and promoting research. advancing education on all aspects of the condition. increasing public acceptance and understanding. representing their national and local interests. providing a point of social contact. Psoriatic Arthropathy Alliance, PO Box 111, St. Albans, Hertfordshire, AL2 3JQ. Reg. Charity No. 1051169. Tel: 0870 7703212 Fax: 0870 7703213 (Telephone support is provided) Email: info@paalliance.org Website: http://www.paalliance.org Return to the Top of Page PSORIATIC ARTHRITIS Arthritis means inflammation of a joint. Psoriatic arthritis is a form of arthritis that occurs in people who also suffer from psoriasis. In the UK, 2-3% of the population develop some form of psoriasis. 10-20% of people with psoriasis develop some form of psoriatic arthritis, with symptoms usually starting between the ages of 30 and 50 (although the condition can occur in teenagers too). In 80% of cases, the arthritis appears after the skin symptoms. In 20% of cases, the joint inflammation comes first. There is no link between the severity of the skin symptoms and the risk of developing arthritis. Over 40% of people with psoriatic arthritis have a family history of the condition. Symptoms As with any type of arthritis, the inflamed joints are tender, swollen and painful. Joints also become stiff after resting, especially after a night’s sleep. Other symptoms include inflamed muscles and tendons, especially around the elbows, wrists and heels. Joints may lose their range of movement and become deformed or locked. Causes A joint forms where two bones come into close contact. Some joints are fixed (e.g. in the skull) whilst in others the bones can move more freely. The bone surfaces in a mobile joint are protected from wear and tear by slippery cartilage and a lubricating fluid (the synovial fluid - produced by the synovial membrane). Most joints are held together by bands of tissue called ligaments. In psoriatic arthritis, the synovial membrane becomes thickened and inflamed. It releases more fluid than normal so that the joint becomes tender and swollen. As inflammation continues, it spreads to the cartilage underneath and may eventually erode the bone. As the tendons are lined and lubricated by synovial membrane, these also become inflamed. Types Asymmetrical oligoarticular - involves one or more joints, especially the knees, fingers and toes that may show sausage-like swelling and redness. Symmetrical rheumatoid-like -involves the small joint of the fingers or toes. Distal interphalangeal (DIP) - involves the last small joint of the fingers or toes. Nail changes are common. Arthritis Mutilans - a rare deforming condition of the joints. Spondylitic - inflammation of the spine and sacroiliac joints. Treatments Aspirin-like drugs (non-steroidal anti-inflammatory drugs) such as ibuprofen reduce the inflammation, but can sometimes make skin symptoms worse and can also cause indigestion or heartburn. They should not be used in those with asthma. Injections of a corticosteroid drug into, or around, a single or painful joint can relieve pain, increase mobility and reduce deformity. Oral corticosteroid drugs are best avoided. They can cause severe relapse of the psoriasis in some people when they are withdrawn. Disease modifying drugs switch off the immune reactions that are causing inflammation and pain. They do not have any immediate effect and may take from 6 weeks to 6 months to work. It is important to avoid pregnancy whilst taking these drugs (effective contraception must be used during treatment). Psoriasis (also see section on Psoriasis) Psoriasis is a long-term (chronic) scaling disease of the skin, caused by the over production of skin cells. The skin shows raised, red patches, which are often covered with dead cells to form fine silvery scales or thick white plaques. Pustules may form when white blood cells (polymorphs) move into the area. Psoriasis is a genetic disease and hence there may be a family history of the condition. For further information contact: Psoriatic Arthropathy Alliance, PO Box 111, St. Albans, Hertfordshire, AL2 3JQ. Reg. Charity No. 1051169. Tel: 0870 7703212 Fax:: 0870 7703213 (telephone support is provided) Email: info@paalliance.org Website: http://www.paalliance.org The Psoriatic Arthropathy Alliance is dedicated to raising awareness and helping people with psoriatic arthritis and its associated skin condition psoriasis. Specifically, the PAA exists to: promote self-help by encouraging good patient compliance. encourage early diagnosis. campaign for better treatments and management of the illness. provide information to initiate appropriate education projects. work with medical and non-medical professionals for the benefit of people with the condition. encourage close working relations with related UK and overseas organisations. Psoriasis Association, Milton House, 7 Milton Street, Northampton, NN2 7JG. Reg. Charity No. 257414. Tel: 01604 711129. Fax: 01604 792894. Email: mail@psoriasis.demon.co.uk Website: www.psoriasis-association.org.uk Return to the Top of Page RAYNAUD'S & SCLERODERMA Raynaud's: Raynaud's is a condition in which the blood supply to the extremities, usually the fingers and toes but sometimes also the ears and nose, is interrupted. It is estimated to affect 3-20% of the adult population and is nine times more common in women. Symptoms During an attack the extremities become first white and dead looking. They may then turn blue and then red and burning. There may be considerable pain, numbness or tingling. The condition can range in severity from minor discomfort, to the onset of ulcers or even gangrene. Progress may be very slow over a period of years and often starts in the very young or early teens. Teenagers are likely to grow out of it in their twenties. Causes These symptoms are due to an intermittent lack of blood in the affected parts when the arteries normally supplying them spasmodically contract. An attack will often be triggered by touching cold objects or exposure to cold of any kind. It seems to be a change of temperature rather than simple cold exposure that triggers an attack, so it can occur in the summer as well as in winter. Emotions, such as anxiety, also play a part, as can smoking. Raynaud's can occur spontaneously or in association with other disorders such as scleroderma, systemic lupus erythematosus or rheumatoid arthritis. People who work with vibratory tools are prone to Raynaud's, which appears to be permanent even when the work with such tools has stopped. However if identified early the symptoms may disappear when the vibration ceases. This condition is known as ‘vibration induced white finger’ and is recognised as an industrial disease eligible for compensation. Treatment Treatment depends on the severity of the condition and for those with a mild condition, stopping smoking, wearing gloves and avoiding the cold may be enough. If the condition is severe, the General Practitioner can advise on the different types of drugs available. These include vasodilator drugs which open up the small blood vessels. Scleroderma The word scleroderma means 'hard skin'. The condition affects the blood vessels, immune system and connective tissue. The skin, usually of the hands and feet, becomes stiff, tight and shiny. The disease is also known as systemic sclerosis, when other parts of the body are affected. It is estimated to affect 3,000 - 3,500 people in the UK. Symptoms In scleroderma the body produces too much of a protein called collagen. This is essential for holding the body together, but too much causes the body to become stiff and unable to function properly. This excess collagen is like scar tissue, it causes thickening and stiffening of those parts of the body it affects. The disease may also affect the connective tissue of the internal organs. There are two major types of scleroderma: Localised scleroderma - which affects isolated areas of skin and the tissues beneath it. This is a relatively mild condition and does not normally affect internal organs. Systemic scleroderma - this affects both the skin and internal organs, including joints, blood vessels, the digestive system, heart, lungs and kidneys. The symptoms include: extreme sensitivity to cold (most people with scleroderma suffer with Raynaud's at some stage in their illness); swelling of the hands and feet, especially in the morning: and the skin of the fingers, toes, trunk and limbs may become tight and shiny. The joints may tighten and bend due to the thickening of the skin. The disease usually starts between the ages of 25 and 55, with women being more often affected than men. Causes The cause is unknown but the condition is neither contagious nor inherited. Treatments Scleroderma is a slow, chronic disorder, usually starting slowly and progressing gradually. Treatment consists of exercise, care of the skin and various drugs to help control the condition or treat complications. Although there is no cure, proper treatment and care can make it possible for people with scleroderma to lead a full life. For further information contact: Raynaud's & Scleroderma Association, 112 Crewe Road, Alsager, Cheshire ST7 2JA. Please enclose an A4 sae. Reg. Charity No. 326306. Tel: 01270 872776. Fax: 01270 883556. Email: webmaster@raynauds.demon.co.uk Website: http://www.raynauds.demon.co.uk Aims of the Association: to promote a greater awareness of Raynaud's, scleroderma and associated conditions. to improve communication between doctors and patients. to put patients in touch with each other in order to exchange ideas and information. to offer advice and support to sufferers and their carers. to raise funds for research and welfare projects. Scleroderma Society, 3 Caple Rd, Harlesden, London, NW10 8AB. Please enclose SAE. Reg. Charity No. 286736. Tel: 020 8961 4912. Email: kim@sclerodermasociety.co.uk Website: http://www.sclerodermasociety.co.uk Return to the Top of Page ROSACEA Rosacea is an inflammatory condition mainly affecting the face. It is hall-marked by episodic flushing of the skin, often lasting for several hours. Some people will periodically develop multiple spots in the affected areas which often become pustular. It is an under-diagnosed condition which may affect up to 10% of the population. There are two peak incidences of rosacea, one in the 20s and one in the 50s, although people of any age may develop the condition. Causes Rosacea can be hereditary and may run in those with Celtic skin types. The flushing attacks may be triggered by a number of different factors. Often these trigger factors will vary considerably from individual to individual. No exclusion list exists that is suitable for everyone. There are, however, many common triggers including: hot (temperature) dairy products food and drinks alcohol caffeine spicy foods stress temperature changes sun wind embarrassment Symptoms After a period of intermittent flushing, the skin becomes persistently red with the development of multiple telangiectasia (tiny broken veins) over the affected area. Experiencing these flushing attacks can be extremely uncomfortable. Attacks can vary from burning to intense pain. Rosacea also causes considerable embarrassment itself. For example, 23% of members of the Acne Support Group with rosacea, who have taken part in public surveys, said that they have been asked whether they had a drinking problem. Treatments Many people believe their facial redness is purely a cosmetic problem and do not seek advice about it. However, it is important to treat this condition early to prevent long term side effects which include: telangiectasia; rhinophyma (enlargement of the nose); persistent oedema of the face (swelling) or eye problems. Treatment should start with identifying possible trigger factors. It could be helpful to keep a diary over a few weeks to help to identify foods which might make the flushing worse. In mild rosacea, topical antibiotics are commonly used. In general, metronidazole is used and needs to be applied sparingly to the whole affected area. In those who fail to respond to topical antibiotics, or those with a more severe disease, systemic antibiotics can be used. Systemic antibiotics tend to have their major impact on the inflammatory lesions, with the flushing tending to respond less quickly. Roaccutane, a hospital-only drug, can also be prescribed to some people, but there are many reported side effects of this treatment, so it should be considered carefully before using. If flushing is a major problem, Clonidine can help to reduce flushing attacks. Rosacea can be confused with acne or seborrhoeic dermatitis, although some people have both rosacea and seborrhoeic dermatitis. Therefore it is important that the condition is diagnosed correctly, so that the most appropriate treatment is administered. It is considered that rosacea is often self-limiting, but it is impossible to predict how long it may last. Many people affected by this skin condition can feel embarrassed and ashamed by their appearance. There are some excellent camouflage creams available from the British Red Cross Skin Camouflage Service, which can help to tone down facial redness and are suitable for both men and women. The key to managing rosacea is to be the one in control of the skin condition, and not the other way around. For further information contact: Acne Support Group, PO Box 9, Newquay TR9 6WG. Reg. Charity No. 1026654. Telephone: 0870 870 2263 Email: alison.dudley@btopenworld.com Website: www.stopspots.org The Acne Support Group provides information and support to those people affected by acne and those people affected by rosacea. Information and services available to members include: a comprehensive information pack. confidential advice. a lively and informative newsletter. Return to the Top of Page SHINGLES & CHICKENPOX Varicella-zoster virus is the virus that causes shingles and chickenpox. Chickenpox is almost universal in the UK with 95% infection rate by age 21. However in other parts of the world, childhood chickenpox is less common leaving immigrants from developing countries susceptible to infection in adulthood with potentially serious consequences. This virus, like the other eight human herpes viruses, remains latent in the body and may reactivate in response to ill-health, stress or trauma to the area. Shingles is the name given to these recurrences and is more likely to affect older people (50% at 85 years). Post herpetic neuralgia (PHN) follows shingles in a rising proportion of older patients: from 50% of 60 year olds with shingles to 70% of those aged 75. Causes and Symptoms Chickenpox (herpes varicella) is very infectious. It is self-limiting and rarely serious in children. Adults may, however, become seriously ill. After an incubation period of 14-21 days a febrile illness will develop followed by typical spots over most of the body. These may develop into ulcers before scabbing over and healing. After the primary infection, herpes viruses remain latent in the body and may reactivate at a later date. Varicella-zoster virus is called shingles if it recurs and the vesicles (spots) are localised, usually on head or chest. Shingles (herpes zoster) cannot be caught although it may be passed to susceptible persons as chickenpox. In shingles, the vesicles and typical pain follow the line of the nerve. Neurological damage can leave symptoms, described variously as excruciating pain, scalded skin, deep aches, tingling, intolerable itching or shooting nerve pains. This post herpetic neuralgia may last months or years. Treatments If the primary infection of chickenpox is severe, and when a shingles occurs in an older patient, one of three antiviral tablets may be prescribed: aciclovir, Famvir or Valtrex. Otherwise all that is necessary is to alleviate symptoms: antipyretics, analgesics, topical anaesthetics as appropriate. Eye infection: Varicella-zoster virus may affect the ophthalmic division of the trigeminal nerve causing pain in, usually, one eye. Diagnosis is by fluoroscein stain. Treatment with antiviral ophthalmic cream as well as antiviral pills is recommended. Eye infection may damage sight or even, rarely, lead to blindness. Because postherpetic neuralgia (PHN) becomes more prevalent with age, over 50s should be started on prescribed treatments to block PHN: a low-dose tricyclic anti-depressant. If PHN develops gabapentin may be added to the drug regimen. Capsaicin cream has been successfully used in several trials. Self-help such as firm bandaging or use of a TENS (transcutaneous electrical nerve stimulation) unit may alleviate the pain. Reported PHN should not be dismissed by doctors as the constant sensations ranging from constant itching to acute pain may render the patient's final years an unendurable misery. Suicides have been recorded. For further information contact: Shingles Support Society c/o Herpes Viruses Association (SPHERE) 41 North Road London N7 9DF Reg. Charity No. 291657 Tel: 020 7607 9661 (office and Minicom V) Fax: on request Website: http://www.herpes.org.uk/shingles.htm The Shingles Support Society is a sub-group of the Herpes Viruses Association (see “herpes simplex”). To receive a 19 page information pack on treating post herpetic neuralgia please send sae (and small donation). The pack includes: Introductory sheets on chickenpox and shingles (2 pages) Tips from other sufferers on dealing with PHN (2 pages) Drug treatments and dosage sheets, fully referenced, for GPs – written by Dr David Bowsher, consultant neurologist, of the Pain Relief Foundation (7 pages) Leaflet on TENS (transcutaneous electric nerve stimulation) (2 pages) Leaflet on dealing with chronic pain (2 pages) A list of names and addresses of people with shingles who would like to communicate with others (2 pages) Return to the Top of Page SKIN CANCER Skin cancer is the second most common cancer in the UK with over 40,500 new cases every year. There are three main types of skin cancer: basal cell carcinoma (BCC), squamous cell carcinoma (SCC) and malignant melanoma (MM). Due to certain similarities basal cell and squamous cell carcinoma are often grouped together and referred to as non-melanoma skin cancer. The prevalence of BCC in the population is 2.1%. BCC is twice as common as SCC. MMs account for 1-in-10 skin cancers. Types and Causes Basal cell carcinoma arises from the cells in the base of the skin and is the most common skin tumour in the UK. It is usually seen in caucasians, particularly those with fair complexion, fair hair and blue eyes. The type of skin affected is almost always hair bearing skin, though occasionally basal cell carcinoma is found on the soles of the feet. Most basal cell carcinomas are slow growing and do not spread. However, if left, they can erode the skin and cause an ulcer, known as a rodent ulcer. Squamous cell carcinoma starts in the surface cells of the skin and is the second most common type of skin cancer in the UK. This is a slow growing cancer but may spread to other parts of the body if left untreated. Like basal cell carcinoma, squamous cell carcinoma tends to occur in caucasians or white skinned people, with more males than females being affected. Malignant melanoma, although not the most common form of skin cancer, does cause the greatest concern as it is curable if found early, but can be very difficult to cure if it has spread into the deeper layers of the skin. MM develops in cells known as melanocytes, which are responsible for the colour of our skin. This type of cancer usually starts in the skin. Rarely it can arise in other parts of the body, such as the eye, the mouth, or in the internal organs. There is strong evidence that ultraviolet (UV) rays from the sun or sun beds can damage the skin and cause cancer. The likelihood of developing a skin cancer increases with age and non-melanoma skin cancer is more common in those over 40 years old. Certain risk factors have been identified. These include people who are fair skinned, burn easily in the sun, are red-haired with freckles, have sunspots (solar keratoses), have had previous skin cancer and those who work or spend long periods outdoors. The risk of developing skin cancer can be reduced by cutting down on exposure to ultraviolet light and increasing protection measures. These include wearing protective clothing, wide brimmed hats and using a strong sun block (minimum SPF 15). Children and young adults who are over-exposed to the sun and suffer blistering or burning are at increased risk of developing a MM in later life. Very rarely, malignant melanoma may be due to a genetic or familial tendency to the disease. Symptoms Both basal and squamous cell skin cancers can appear anywhere on the body but are more likely to appear on exposed skin, especially the face, neck, arms, hands and lower legs. They can appear in a variety of forms: a small lump on the skin (which is smooth and pearly or waxy in appearance, or which bleeds, crusts and does not heal, or is wart-like in appearance); a flat red spot or a firm red lump. Most MMs start in normal skin. Others may develop in existing moles. If it does develop from a mole, changes include: a change in size; altered shape; change in colour; itching, crusting or bleeding. People should be encouraged to report any unusual marks on the skin which last more than a few weeks or an existing mole which shows any of the above signs. Many people feel overwhelmed when told they have a diagnosis of skin cancer. The impact can be particularly devastating if the cancer is on a prominent part of the body, such as the face, neck or arms/hands. The prospect of potentially disfiguring treatment can be hard to cope with. People in this situation need accurate information and sensitive support to help them understand what is happening. Treatments Treatment options include: surgery; electrocautery; cryosurgery; lymph gland removal; radiotherapy; chemotherapy and, immunotherapy (for MM). These treatments may be used alone or in combination. Most people with non-melanoma skin cancer are cured, whilst the prognosis for MM depends on the depth of the cancer in the skin. It is important to report a MM early. For further information contact: Cancer Research UK PO Box 123 London WC2A 3PX Tel: 020 7242 0200 www.cancerresearchuk.org Specialist information nurses: 020 7269 3142 Patient information website www.cancerhelp.org.uk Registered charity number: 1089464 Cancer Research UK is dedicated to research on the causes, treatment and prevention of cancer. Wessex Cancer Trust’s Marc’s Line, Marc’s Line Resource Centre, Dermatology Treatment Centre, Level 3, Salisbury District Hospital, Salisbury, SP2 8BJ. Tel: 01722 415071. Website: http://www.wessexcancer.org/ (part of the Wessex Cancer Trust Cancer Information Network) Return to the Top of Page TUBEROUS SCLEROSIS T uberous sclerosis is an autosomal dominant disorder of variable expression, characterised by extra tissue growths, hamartomas, in almost any organ of the body. Hamartomas include cortical tubers and subependymal nodules in the brain, retinal phakomas, renal cysts and angiomyolipomas, cardiac rhabdomyomas, pulmonary cysts and a variety of cutaneous stigmata - hypomelanic macules, facial angiofibroma, ungual fibroma, shagreen patches and forehead plaques. It is associated with learning disability (in up to 50% of cases), epilepsy (60-70%) and autistic traits (50%). Up to 90% will have some skin signs, and in some cases these will be the only clinical feature of the condition. The condition affects between 1-in-8,500 to 1-in-10,000 people. Causes Tuberous sclerosis is caused by a defect in cell growth regulating genes, either on chromosome 9 or on chromosome 16. Although approximately two thirds of cases are sporadic, it is essential that genetic counselling is offered to families after diagnosis because of the variability of the disorder. A diagnosis is presently confirmed by clinical and radiological investigations. DNA tests, including pre-natal testing, are now possible for some families. Symptoms Tuberous sclerosis can present at any age, with symptoms ranging from seizures or cardiac arrhythmias in infancy, to the later appearance of facial angiofibromas or renal angiomyolipomas. Ensuing investigations may subsequently reveal hypomelanic macules (90%), which fluoresce under ultra-violet light, calcified subependymal nodules (80%), which show up on a CT brain scan, and, in neonates, cardiac rhabdomyomas (60%). Life expectancy is generally good, even for those with severe learning disability. Infrequently, death can occur from brain tumours or kidney disease, but most people have good long-term prospects if monitored appropriately for signs of serious problems. Skin signs which present from birth onwards are hypomelanic macules (90%), which can repigment in adult life, and smooth, fibrous forehead plaques (25%). Shagreen patches (40%), thickened discoloured skin or nodular lesions, are more likely to develop later in the lower lumbar area. Facial angiofibromas (85%) can appear at any age from two years upwards, starting as tiny red dots and developing during adolescence across the nose, upper cheeks, and particularly in the chin and nasolabial folds. This rash used to be misleadingly called adenoma sebaceum. Ungual fibromas (50%) can grow from the nails of both hands and feet, from late adolescence onwards causing grooves in the nails even if the lesion cannot be seen. Skin tags occur in 30% of people affected by the condition, and can be profuse at the back of the neck, across the shoulders, in the groin or on the testes. Treatments Since there is no cure for tuberous sclerosis, treatment has to be symptomatic. Epilepsy is treated with drugs (or occasionally surgery); raised intracranial pressure can usually be treated by surgical removal of the tumour and/or the insertion of a shunt; renal problems may require embolisation of an angiomyolipoma or more rarely partial nephrectomy, dialysis or transplantation; symptomatic lung disease may respond to treatment with progesterone; and cardiac problems only rarely require medical treatment. Behavioural difficulties (including hyperactivity, autistic features, sleeping problems and hallucinations) are often the most troublesome for families and frequently require appropriate intervention. Of the various skin manifestations associated with tuberous sclerosis, there are three which may benefit from treatment. The forehead fibrous plaques and similar growths on the head can sometimes enlarge considerably in late adolescence and require plastic surgery. The ungual fibromas can be removed either by laser or diathermy (although they usually regrow whatever treatment is used). However it is the facial angiofibroma which cause the most psychological distress to patients and these can also bleed profusely if rubbed. These angiofibromas benefit most from the laser treatments, which are currently thought to be the most effective and least traumatic treatments. The argon and pulsed dye lasers have more success on the vascular lesions, whilst the carbon dioxide laser is more successful where there is a greater fibrous component to the lesions. For further information contact: Tuberous Sclerosis Association, PO Box 9644, Bromsgrove, Worcestershire B61 0FP. Reg. Charity No. 1039549. Tel: 01527 871898. Email: support@tuberous-sclerosis.org Website: http://www.tuberous-sclerosis.org The Tuberous Sclerosis Association has three main aims: supporting people with tuberous sclerosis and their families or carers, providing information and advice on the disease and its management. providing education, publicity and information to promote an understanding and awareness of the problems encountered by the condition. funding research into the causes and management of tuberous sclerosis. Return to the Top of Page VITILIGO Vitiligo is one of the most common skin diseases, and yet the vast majority of people have never even heard the name. Probably because vitiligo is not life threatening, statistics on its prevalence are not routinely collected. On the basis of various clinical studies around the world, it is generally estimated to affect 1-4% of all races and populations. This implies that over half a million people in the UK alone have vitiligo. Symptoms and Causes Vitiligo can affect anyone of any age and either sex. In those who have the condition, patches of skin turn white, and in some cases their hair can also lose its colour in patches. Although it has no effect on the patient’s general physical health, the white patches usually increase over time , producing further patches with patches joining together to form large areas of totally white skin and are highly vulnerable to sunburn, even when only briefly exposed to mild sunshine. On exposed areas such as the face and hands it can be very disfiguring, especially on dark or tanned skin. Many people who have vitiligo find it socially and psychologically devastating, and can lose their self esteem and self confidence. The causes are not yet properly understood, but there is growing evidence which indicates that the disease is genetic in origin in most cases. There are other skin conditions, particularly fungal infections, which can easily be mistaken for vitiligo. If there is any doubt about the diagnosis, a referral to a hospital dermatology (skin) clinic is advised. Treatments An outright cure has not yet been found, but there are recognised National Health Service (NHS) treatments which can sometimes bring colour back into the white patches or at least control their spread, although there is no guarantee that they will work in any individual case. One of these treatments is the use of steroid creams on the affected areas at the onset of the disease. This can sometimes stop the spread of the patches or, even restore some of the lost colour. This treatment is usually given only for a limited period under medical supervision. Another NHS treatment involves the use of ultraviolet light. One of these treatments, known as PUVA (psoralen + UVA), can encourage the natural colour to come back within the white patches. This treatment involves the use of drugs called psoralens, which make the skin sensitive to light. The skin is then exposed to ultra-violet A light . In the UK, PUVA is normally available only as a hospital outpatient treatment, and it should always be supervised by a dermatologist (skin specialist). A more recent development is the use of narrow-band ultraviolet B light This has the advantage that no drugs need to be taken, and the repigmentation is often more successful with this method than with PUVA. In the UK this treatment is usually only available in hospitals under the supervision of a dermatologist. A further development in research, has shown that psychological counselling using cognitive behavioural therapy can both help the patient to recover his/her self-esteem and can, in some cases, help lost colour to return to the white patches. Complementary treatments, including herbal and homoeopathic remedies, can be very expensive. They may sometimes work, but there is no proof of this and there could be unforeseen side effects. The patient should be cautious and should never take any medicine or pills without knowing what they contain. It is important to inform the General Practitioner about any treatments undertaken, and to make sure that the complementary practitioner consulted is properly qualified and registered with a recognised professional body. Anyone who claims to offer a ‘cure’ should be avoided. For those who are troubled by their appearance, concealing the white patches may improve self confidence. There are cover creams which are specially designed for this purpose and are suitable for use by men, women and children alike. Some of these, classified by the NHS as ‘borderline substances’, can be prescribed by doctors. The Skin Camouflage Service of the British Red Cross has a nationwide network of clinics which advise on the use of cover creams. Some people prefer to use self-tanning creams (sometimes called fake tans), which are available over the counter at most chemists or beauty counters. It is important to protect the white patches from burning in the sun. These patches are wholly or partly lacking in the pigment which is the skin’s natural protection against sunburn. Allowing the skin to burn can encourage the vitiligo to spread. The patches should be protected from the sun by clothing, or by the use of a high factor sunblock. Some brands of sunblock, which have a sun protection factor of 25 or above are classified as borderline substances and can be obtained on prescription. Children and young people Children with vitiligo cope best if parents answer their questions about their condition truthfully and simply. They need to be reassured that it will not affect their family’s feelings towards them. They can have problems with bullying and teasing at school, which may be overcome by explaining vitiligo to teachers and enlisting their help. For further information contact: Vitiligo Society, 125 Kennington Road, London, SE11 6SF. Reg. Charity No.1069607. Tel: Freephone 0800 018 2631. Fax: 020 7840 0866. Email: all@vitiligosociety.org.uk Website: www.vitiligosociety.org.uk The Vitiligo Society defines its mission as promoting a positive approach to living with vitiligo. It aims to: offer support and understanding to people with vitiligo and to their families. offer advice on how to cope with the condition. promote and fund research with the objectives of establishing the causes of vitiligo and finding a safe and effective treatment. campaign for a better understanding of vitiligo among the medical profession and the general public. gather and distribute information about the condition. Return to the Top of Page XERODERMA PIGMENTOSUM Xeroderma Pigmentosum (XP) is a rare genetic disorder that may cause extreme sensitivity to the sun's ultraviolet rays. Unless patients with XP are protected from sunlight, their skin and eyes may be severely damaged. This damage may lead to cancers of the skin and eye. XP has been identified in people of every genetic group all over the world. There are about 100 confirmed cases in the UK. Causes Ultraviolet light damages the DNA in cells and disrupts normal cell functioning. DNA (deoxyribonucleic acid) within our genes contains all the coded information needed to direct cell functions. Two factors combine to cause the abnormalities in XP. Firstly, a person inherits traits from each parent which, when combined, lead to an unusual sensitivity to damaging effects of ultraviolet light. Secondly, exposure to the sun, which contains ultraviolet light, leads to changes in the skin and eyes. Damaged DNA is repaired by the DNA repair system. But the DNA repair systems of people with XP do not function properly. As a result, un-repaired DNA damage builds and causes cancerous cell changes or cell death. Symptoms Many people with XP get unusually severe sunburn after a short period of sun exposure. The sunburn may last much longer than expected, perhaps for several weeks. This type of sunburn will usually occur during a child's first exposure, and it may be a clue to the diagnosis of XP. However, some people with XP do not burn more easily and the disease will be undetected until unusual skin changes appear over time. Most patients with XP develop freckles at an early age. Continued sun exposure will lead to further changes in the skin, including irregular dark spots, thin skin, excessive dryness, rough-surfaced growths and skin cancers. These skin changes will resemble those of elderly people who have spent many years in the sun. In people with XP, the changes caused by sun damage often begin in infancy and almost always before the age of 20. The eyes of a person with XP are often painfully sensitive to the sun and may easily become irritated, bloodshot and clouded. Non-cancerous and cancerous growths on the eyes may occur. Treatments There is no cure for XP, but much can be done to prevent and treat some of the problems it causes: Protection from ultraviolet light, by a combination of physical and chemical means. These include sun avoidance, shade, clothing (including hats), optical filtration and sunscreens. Frequent skin and eye examinations. Prompt removal of cancerous tissue. Neurological examination in some cases. Text extracted from: 'Understanding Xeroderma Pigmentosum' published by US Department of Health & Human Services (by permission). For further information contact: XP Support Group, 2 Strawberry Close, Prestwood, Great Missenden, Bucks., HP16 0SG. Reg. Charity No. 1075302. Tel: 01494 890981. Fax: 01494 864439. E-mail: info@xpsupportgroup.org.uk Website: http://xpsupportgroup.org.uk/ The XP Support Group is a charitable trust set up to continue the work of the Xeroderma Pigmentosum Society Inc., a non-profit organisation based in New York State, USA. Parents of children with XP set up both the XP Society (USA) and the XP Support Group (UK). The XP Support Group is independent of the XP Society. The aims of the Support Group are: to relieve the needs of people with Xeroderma Pigmentosum and UV-related conditions and their families; to advance the education of the public in Xeroderma Pigmentosum; to promote research into Xeroderma Pigmentosum. To achieve these aims the Group will endeavour to:  raise funds for the XP Research fund set up by the XP Society; assist families to attend Camp Sundown (a night-time camp held once a year in the USA) or respite in a protected environment. Our respite home is at St Katharine’s, Frieth in Buckinghamshire, and is where we hold our annual night-time camp called the “Owl Patrol”; give grants for UV protective products; raise public awareness by means of an education campaign. Return to the Top of Page info@derma-products.com 1-866-803-4842 [Toll Free] Information provided by Dermalogica. 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