Cutaneous aspects of Refsum's disease.
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Biomedical subjects
Publications and source records attributed to J J Cream.
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Curettage of skin lesions was not followed by bacteraemia in 22 patients. The risk of bacterial endocarditis after curettage and other minor skin surgery is small but should not be overlooked in those with a prosthetic heart valve, a history of other cardiac surgery, a previous episode of infective endocarditis, drug addiction, diabetes, alcoholism, immunosuppression, or renal failure--especially where the skin lesion might be infected.
A case of generalized histiocytoma cutis occurring in an atopic and mimicking widespread molluscum contagiosum is described. Immunochemical staining showed that the lesions were positive for factor XIIIa.
The endonuclease digest patterns of viral DNA from 48 genital and 45 non-genital molluscum contagiosum virus (MCV) lesions were examined. The overall ratio of MCVI to MCVII was 3.23:1. There was no predominance of either MCV type in genital lesions. No obvious morphological differences were seen between MCVI and MCVII lesions. MCVII was not found in any patient under 15 years old.
We report eight patients with ulcerative colitis who have subsequently developed pemphigoid. Four patients were investigated to determine the pattern of pemphigoid antibody staining on whole and chemically split skin and oral mucosa to distinguish between pemphigoid and epidermolysis bullosa acquisita. The findings were suggestive of pemphigoid with a predominantly epidermal pattern. Three patients were studied for the presence of antibodies against the colon (which were absent). The relationship between pemphigoid and ulcerative colitis is discussed in relation to a case-control study.
Restriction endonuclease analysis of molluscum contagiosum virus DNA revealed two subtypes. In a study of 46 isolates from 41 patients, some with no other disorder and some with atopic dermatitis, the ratio of MCV I isolates to MCV II was 34:12. Multiple clustered lesions removed at the same time from an individual patient yielded only one type of MCV. Lesions induced by MCV I or MCV II were indistinguishable on the basis of size and form. Neither subtype was associated exclusively with lesions at certain sites or with other clinical features. Heterogeneity of DNA restriction endonuclease cleavage patterns amongst isolates of the same subtype was observed, this being greatest for MCV II.
Galactosaemia has been suggested as a contributory factor in the pathogenesis of some presenile and senile cataracts. To assess whether galactosaemia plays any part in the development of atopic cataracts a galactose tolerance test was carried out in eight atopic dermatitis patients whose cataracts had appeared between the ages of 12 and 39 years. In seven patients a normal result was obtained and in one the result was just above normal. Impaired galactose tolerance appears to have no role in the pathogenesis of atopic cataract.
Abnormal levels of the essential fatty acids (EFA) in plasma phospholipids have been reported in atopic dermatitis. We have compared EFA levels in 10 patients with atopic cataracts and 29 atopic dermatitis patients without cataracts and found no significant differences, with the exception that higher levels of adrenic acid occurred in those with cataracts.
Sera from 91 patients with hereditary angioedema were screened for thyroid antibodies. The results for the 77 patients more than 17 years old were compared with previously published data for the prevalence of thyroid disease in a large community (Whickham). Of the female patients with hereditary angioedema, the prevalence of thyroglobulin antibodies (TGA) was 14.0%, higher than the expected 3% (p less than 0.001). The prevalence of thyroid microsomal antibodies (TMA) was 20%, also higher than the expected 7.6% (p less than 0.01). The age distributions of the females in both groups differed: in the group with hereditary angioedema there was a greater proportion of younger patients which should have resulted in a lower prevalence of thyroid antibodies. Adjusting for related patients with hereditary angioedema, there was still an increased prevalence of TGA (p less than 0.01) and TMA (p less than 0.01).
Distinctive patterns of skin disease other than Kaposi's sarcoma have been in patients with AIDS, in others with persistent generalized lymphadenopathy (PGL) and in a group at high risk of developing AIDS. We found a chronic acneiform folliculitis on the face, back, chest and buttocks, extensive cutaneous fungal infections and a striking neck and beard impetigo. These skin diseases were not present in asymptomatic male homosexual control subjects, 32% of whom were found to have antibodies to human T cell lymphotropic virus type III (HTLV-III). We regard these dermatoses as early warning signs of AIDS.
The effect of oral spironolactone (200 mg daily) on acne vulgaris has been studied in 21 women in a randomized, placebo-controlled, double-blind crossover study using 3 month treatment periods. Compared with placebo, spironolactone produced significant improvement as assessed by subjective benefit (P less than 0. 001), number of inflamed lesions (P less than 0 . 001) and by an independently evaluated photographic method (P less than 0 .02). There was a fall in sex hormone binding globulin but no significant changes in plasma testosterone and derived free testosterone. Initial plasma androgen levels were no higher in responders than in non-responders, nor did oral contraceptive use appear to affect clinical response. Spironolactone is a useful alternative therapy for women with acne vulgaris.
A 49 year old man with systemic sclerosis developed ischaemia of his extremities with massive peripheral gangrene. High levels of circulating cryofibrinogen were detected in the plasma and the significance of these findings is discussed.
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In a consecutive series of thirty-six male and female patients referred with severe acne, the effect of 3 months' treatment with placebo or spironolactone (50-200 mg daily) on sebum excretion and clinical and endocrine status was evaluated double-blind. Twenty-six patients completed the study. Abnormal free androgen indices were found in 27% of the original nineteen female subjects. Spironolactone reduced sebum excretion in all female subjects, but there was no correlation between sebum response and androgen status. The clinical response was dose-dependent, with maximum subjective and objective benefit when spironolactone doses of 150-200 mg were used.
An extremely rare case of primary extragenital granuloma inguinale affecting the axillae is reported. Clinical, histological, and electron microscopical features of the disease are described, and the rarity, absence of genital lesions, and consequent difficulty in diagnosis are emphasised.
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