Diclofenac-induced pseudoporphyria; an under-recognized condition?

Pseudoporphyria is a photodistributed bullous disorder that is clinically and histologically similar to porphyria cutanea tarda (PCT), but without abnormal porphyrin biochemistry. Renal failure, dialysis, excessive ultraviolet A and medications, particularly nonsteroidal anti-inflammatory drugs (NSAIDs), have been associated with pseudoporphyria. We report a case of diclofenac-induced pseudoporphyria in a man with psoriatic arthritis. To our knowledge, this is only the second reported case of pseudoporphyria associated with diclofenac. Diclofenac has hitherto been considered by many dermatologists as a safe alternative in NSAID-induced pseudoporphyria.

Advised best practice for the use of emollients in eczema and other dry skin conditions.

The recent Dermatological Care Working Group report highlighted important deficiencies in the dermatology service in the UK and recommended that care should move closer to the patient. The report stated that 'expert patients' could become 'sharers in their care' and are best placed to improve their own self management. One area that could benefit greatly from increased patient education and participation is the use of emollients. Emollients are frequently prescribed for patients with eczema and other dry skin conditions. Although the benefits of emollient therapy are widely accepted, prescribing practices vary considerably, often according to physicians' individual preferences. Patients can receive confusing or conflicting treatment advice, leading to frustration, non-compliance, and difficulty in following an effective regimen. To promote the effective use of emollients it is important for patients and health professionals to understand the functions of the skin and the principles of emollient use and application. We propose a set of simple guidelines for emollient therapy in eczema care to improve day-to-day management by health professionals in the community and to promote consistent practices by patients. These guidelines form the ABC dry skin and eczema management programme supported by the National Eczema Society and accredited by the British Skin Foundation.

Pyoderma gangrenosum of the eyelid and acute rhinosinusitis.

Pyoderma gangrenosum (PG) is a destructive, necrotizing, noninfective ulceration of the skin. Periorbital PG is extremely rare, and may progress, with eventual loss of the eye. We report a 47-year-old woman who presented with a right periorbital swelling of 8 days duration and concurrent acute rhinosinusitis. A clinical diagnosis of right periorbital PG was made, and treatment instituted with intravenous methylprednisolone and antibiotics, resulting in rapid resolution of the condition. The clinical features in this case, together with the past history of histologically confirmed PG, enabled a rapid diagnosis to be made, with early administration of treatment resulting in minimal scarring. The clinical picture could easily be confused with periorbital cellulitis, and this case demonstrates the importance of considering the differential diagnoses of periorbital swelling.

Lichen myxoedematosus with associated cardiac abnormalities.

We describe a 42-year-old woman who developed lichen myxoedematosus. Twenty years after the onset of the disease she became breathless and hypertensive, and an echocardiogram showed a mass on the mitral valve, which was thought to be a mucin deposit. Her hypertension was resistant to treatment with combination antihypertensives. To our knowledge, this is the first report to link lichen myxoedematosus with a valvular mucinous mass. This case also demonstrates the slow clinical progression of the disease over 20 years.

Association of multiple familial cutaneous leiomyoma with a uterine symplastic leiomyoma.

We describe a patient who has familial cutaneous leiomyoma in association with a symplastic uterine leiomyoma. This association has not been described previously.

The perils and pitfalls of penile injections.

We describe a 45-year-old man who presented with an indurated penile nodule following self-injection of acyclovir tablets which he had dissolved in hydrogen peroxide solution. This is a hazardous procedure which may be complicated by permanent deformity and functional disability due to the irritant nature of the tablets' constituents and their propensity to cause foreign body reactions in the skin.

A multidisciplinary vulva clinic: the role of genito-urinary medicine.

OBJECTIVES: To characterise the new patient referrals to a combined vulva clinic and to assess the role of genitourinary services within the clinic. METHODS: A case note review of all new patients attending a monthly, multidisciplinary vulva clinic over a 12-month period. RESULTS: The mean age of the 135 women was 43 years (range 18-86 years). The majority of patients, 64 (47%), were referred by their general practitioner (GP). Using nurse and physician triage 85 (63%) patients were seen by a dermatologist, 55 (41%) by a genitourinary medicine physician, 38 (28%) by a gynaecologist and six (4%) by a psychosexual physician. Fifty-one (38%) women required a consultation by at least two specialties. Itch was the most frequent presenting symptom (70%) and 59 (44%) women had experienced symptoms for between 6 months and 2 years. A previous STD screen had been performed in only 57 (42%), which was negative in 45 (79%). The most frequent initial clinical diagnoses were lichen sclerosus (35, 26%), vaginal candidiasis (21, 16%), vulvodynia (16, 12%), lichen simplex chronicus (13, 10%) and Bowenoid papulosis (13, 10%). Thirty-eight (28%) women had microbiological investigations revealing 13/135 (10%) had vaginal candidiasis and two (2%) bacterial vaginosis, all symptomatic. A biopsy was performed in 32 (24%) confirming the initial diagnosis in 20 (63%) cases. Treatment was initiated in 101 (75%) women: 62 (46%) were prescribed steroid cream, 46 (34%) emollient cream and 22 (16%) treatment for candida infection. Fifty-three (39%) women received more than one treatment. 94 (70%) patients were followed-up in the vulval clinic, five (4%) in the genitourinary clinic and 12 (9%) by their GP. CONCLUSIONS: Despite having genitourinary symptoms less than half the patients had been tested for infection prior to attending the clinic. More than a third of the patients, 46 (34%), were diagnosed with a genitourinary infection. There is a significant role for genitourinary services in the diagnosis, management and ongoing care of patients in a vulva clinic.

Pyoderma gangrenosum associated with Takayasu's arteritis responding to cyclosporin.

A 33-year-old caucasian woman with pyoderma gangrenosum associated with Takayasu's arteritis responded to treatment with cyclosporin. This patient is unusual in that both ulcerative and vesiculopustular forms of pyoderma gangrenosum were present. This has not previously been reported with Takayasu's arteritis.

Itchy folliculitis and human immunodeficiency virus infection: clinicopathological and immunological features, pathogenesis and treatment.

The predominant itchy folliculitis associated with human immunodeficiency virus (HIV) infection appears to be an eosinophilic folliculitis (EF). This is characterized by lytic degeneration of sebaceous glands and an inflammatory infiltrate in which eosinophils and CD8+ T lymphocytes predominate. All patients have low CD4 counts and present late on in their HIV disease. Lesional distribution is mainly truncal, with a significant proportion also having facial involvement. Our prospective survey has shown that it is impossible to differentiate clinically between infective folliculitis and EF, and we recommend therefore that all cases are biopsied. We review the clinicopathological and immunological aspects of HIV-associated itchy folliculitis, in particular HIV-associated EF as well as current theories on pathogenesis and treatment. We suggest that HIV-associated EF is an autoimmune disease with the sebocyte or some constituent of sebum acting as the autoantigen.

Severe vulval apocrine acne successfully treated with prednisolone and isotretinoin.

Apocrine acne, otherwise known as hidradenitis suppurativa, is a chronic inflammatory scarring disease affecting the apocrine gland-bearing skin. We present a case of a 34-year-old woman with severe vulval apocrine acne who was successfully treated initially with prednisolone and then maintained on long-term isotretinoin. This case indicates that long-term treatment with isotretinoin may be more successful than the usual 4-6 months treatment regime.

HIV associated eosinophilic folliculitis--differential diagnosis and management.

Eosinophilic folliculitis (EF) is a chronic, intensely pruritic condition of unknown pathogenesis that causes marked morbidity in those HIV patients whom it affects. There is a wide differential diagnosis of itchy skin conditions in HIV which are amenable to different treatments. It is therefore essential to take a biopsy of each suspected case and examine multiple sections of the biopsy to confirm or refute a diagnosis of EF. Treatment of EF can be difficult but we hope that by suggesting a rational approach to this and considering possible therapeutic options more patients may be helped with this troublesome dermatosis.

Use of topical cyclosporin in oral pemphigus.

The use of systemic cyclosporin in the treatment of pemphigus is well recognized. Oral lesions in pemphigus vulgaris are common but can be very difficult to treat. Short use of topical cyclosporin has been reported in a few cases of oral pemphigus. We report the successful use of topical cyclosporin for several years in a patient with debilitating oral pemphigus in whom systemic immunosuppressive agents and topical steroids had been ineffective and in whom cyclosporin was used not only to induce but also to maintain remission.