One hundred and thirty-seven patients with Darier's disease and 66 patients with Hailey-Hailey disease completed the Dermatology Life Quality Index (DLQI). The average DLQI score for Darier's disease was 5.89 (19.6% of the maximum score), median = 4, quartiles: 1.9. The average DLQI score for Hailey-Hailey disease was 6.06 (20.2% of the maximum score), median = 5, quartiles: 2.9. The DLQI score did not correlate with the physician's assessment of clinical severity in both diseases, thus emphasizing the importance of remembering the potential for handicap even in patients with disease that seems to be mild. The domains of the questionnaire with the highest mean scores in both diseases were for questions relating to symptoms and feelings. Domains relating to personal relationships and work obtained low mean scores even in severe disease. It is encouraging that most patients with Darier's disease and Hailey-Hailey disease maintain good relationships at work and home and seem to be able to adapt to their skin disease.
Epidermodysplasia verruciformis (EV) is a rare inherited condition in which there is widespread infection with human papillomavirus (HPV). Patients have a high risk of developing squamous cell carcinoma and Bowen's disease on sun-exposed sites. We describe a Jamaican man with the typical clinical and histopathological features of EV.HPV 8, 24 and a subtype of HPV 38, along with a novel HPV sequence most closely related to HPV 9 have been detected in his skin lesions. Although skin tumours are rare in black patients with EV and he has lived in a temperate climate most of his life, several of the lesions showed bowenoid atypia and he is at risk of developing invasive cutaneous malignancies.
Summary We report the onset of dermatomyositis in a woman pregnant 38‐weeks who subsequently delivered a healthy infant. The disease improved rapidly following delivery. The association of dermatomyositis with pregnancy is unusual, and fetal outcome may be adversely affected.
A 48-year-old man presented with a history of recurrent erythematous lesions on the trunk and limbs. Examination suggested a tixed drug eruption and this was conlirmed by oral challenge with paracetamol. The histology was unusual as il showed a leucocytoclastic vasculitis. Patients may have difficulty avoiding the offending drug because many drugs bought over the counter have similar names but difterent constituents. In addition, il may be difficult to persuade the patient that a specific drug is responsible for their skin problem. Case reportA 4S-year-o!d man presented with a history that, on four occasions in the previous 20 months, he had developed an uncomfortable, red rash. The rash recurred every 4-6 months. Forty-eight hours prior to the development ofthe rash, on each occasion, he had noticed 'tlu-like symptoms for which he had taken paracetamol or Beecham's Powder capsules (which contain paracetamol, caffeine and phenylephrine hydrochloride). The rash recurred at the same sites, lasted 2 days, and faded to leave residual areas of hyperpigmentation. Between those episodes, he denied ingestion of any medication.Examination at an acute stage showed well defined erythematous macules and plaques scattered over the trunk and limbs (I'ig, 1). The rash appeared purpuric, particularly on the feet and ankles, and there was some exfoliation but no blistering. One week after their onset the lesions faded to leave macular hyperpigmentation.The differential diagnosis included urlicarial vasculitis and a fixed drug eruption. Routine haematological and biochemical studies were normal. Tests for antineutrophil cytoplasmic antibody and extractable nuclear antigen were negative. Serum complement levels were normal. A skin biopsy was taken from a fresh erythematous lesion and showed on histology a leucocytociastic vasculitis affecting the superficial capillaries, with a mixed perivascular infiltrate containing lymphocytes, neutrophils and eosinophils (Fig, 21. There was some pigmentary incontinence but no basal cell degeneration or keratinocyte necrosis. Iron stains were negative.An oral challenge test was performed with 1 g para-790 cetamol. Thirty minutes after ingesting the drug, previously asymptomatic hyperpigmented macules became red and uncomfortable. He had no associated systemic symptoms and the rash faded over the next 4 days to leave hyperpigmented macules. He has no further problems since avoiding paracetamol. DiscussionOur patient has had a fixed drug eruption to paracetamol, and has the unusual histological linding of a leucocytoclastic vasculitis. Although paracetamol is well documented as a cause of a fixed drug eruption, only two cases were reported to the Medicines Control Agency (MCAl between 1963 and 1994. This probably refiects under-reporting. Figure L Erythematous macules present on the legs. (C; 1995 British Associalion of Dermatologists
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