D. Burrows

Subcorneal pustular dermatosis and IgA paraproteinaemia: response to both etretinate and PUVA

Summary A non‐insulin dependent male diabetic is reported with subcorneal pustular dermatosis associated with intraepidermal IgA deposits and a benign IgA paraproteinaemia. Treatment with dapsone and etretinate was reasonably effective, but etretinate had to be discontinued due to the development of diffuse idiopathic skeletal hyperostosis. His subcorneal pustular dermatosis subsequently flared and was troublesome for 2 years until he was commenced on PUVA, with excellent response.

Seborrhoeic eczema—a disease entity or a clinical variant of atopic eczema?

One hundred and thirty‐eight children seen at the Dermatology Clinic between 1969 and 1972 were reviewed. The study comprised two groups—a study group of 76 children diagnosed as having seborrhoeic eczema, and a group of 62 children seen over the same time period at the same clinic and diagnosed as having atopic eczema. The children were examined and a family history obtained with particular reference to past or present history of atopic diseases. Serum IgE, blood eosinophil count, pulmonary function tests and skin prick testing to various allergens were measured in the two groups. In the seborrhoeic group, 19% had eczema, 20% had abnormal pulmonary function clinically; 25% had a raised eosinophil count, 44% a raised IgE level, 89% positive skin prick testing, and 52% a peak flow less than 80% of predicted value. These results indicate a closer than expected association between infantile seborrhoeic eczema and atopic disease.

Dermatological presentation of disease associated with antineutrophil cytoplasmic antibodies: a report of two contrasting cases and a review of the literature

Summary Antineutrophil cytoplasmic antibody (ANCA)‐associated disease (AAD) constitutes a pathological disease spectrum of a necrotizing vasculitis of small‐ and medium‐sized vessels, extravascular granuloma formation. and necrotizing and crescentic glomerulonephritis. and also a clinical disease continuum which ranges from renal‐limited disease to a widespread systemic vasculitis. including Wegeners granulomatosis and microscopic polyangiitis. In the latter, circulating ANCA are an aid to diagnosis and also may play a pathogenic part. Two contrasting patients with AAD are described. both of whom presented primary with dermatological features. These included a cutaneous purpuric vasculitis. orogenital ulceration. infarction of the fingertip, and pyoderma gangrenosum‐like ulceration. These cases will familiarize dermatologists with both the concept and dermatological features of AAD.

Erythropoietic protoporphyria, transfusion therapy and liver disease

A 28‐year‐old man who had suffered from erythropoietic protoporphyria since infancy was referred because of worsening photosensitivity. Conventional therapy with β‐carotene, terfenadine and topical sunscreens was ineffective or not tolerated, and he was treated with transfusions of washed packed cells. Unexpectedly, his photosensitivity deteriorated further, his whole blood protoporphyrin levels doubled and he developed abnormal liver function tests. This is the first report of such an adverse response to blood transfusion therapy for erythropoietic protoporphyria and may have been related to subclinical hepatitis or the increased iron load associated with blood transfusion.

LETTER‐SIWE DISEASE REPORT OF SIX CASE

SUMMARY.— Six cases of Letterer‐Siwe disease occurring in a population of 1,500,000 over a period of 10 years are described. Five have died and 1 survives 2 years after cessation of therapy and 4 years after diagnosis. Of those who died, in 2 the terminal event was measles infection, in 1 surgical emphysema consequent on rupture of a lung cyst and in 2 death followed progression of the disease.

Laryngo-onycho-cutaneous syndrome associated with familial benign hypercalcemia

A Pakistani boy had chronic ulceration of the cheeks, generalized nail dystrophy, ulceration and hypergranulation of the nail beds, conjunctiva, and vocal cords, and dental enamel hypoplasia. These features are consistent with laryngo-onycho-cutaneous (LOCS) syndrome or Laryngeal and Ocular Granulation tissue in children from the Indian subContinent (LOGIC) syndrome. Both he and his elder brother had elevated levels of serum calcium consistent with familial benign hypercalcemia. Laryngo-onycho-cutaneous syndrome is a newly recognized condition and its association with familial benign hypercalcemia has not been previously reported.