Deep and acute vulval ulceration. Case report.
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Biomedical subjects
Publications and source records attributed to R A Marsden.
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Linear IgA disease of adults, chronic bullous disease of childhood, and the rare childhood cicatricial pemphigoid currently are regarded as separate clinical entities despite their many shared features. All are sulfone-responsive subepidermal bullous diseases associated with linear IgA deposition at the basement membrane zone. In this paper we present a long-term study of 25 cases of adult linear IgA disease, 25 cases of chronic bullous disease of childhood, and four cases of childhood cicatricial pemphigoid, which has revealed further similarities among all three groups. The morphology and distribution of the cutaneous and mucosal lesions were similar; mucosal involvement was present in 80% of patients with adult linear IgA disease, 64% of those with chronic bullous disease of childhood, and 100% of those with childhood cicatricial pemphigoid, and ocular scarring affected patients in all groups. Remission occurred in 64% of those with chronic bullous disease of childhood (the disease was active in 12% after puberty), 48% of those with adult linear IgA disease, and in no cases of childhood cicatricial pemphigoid. HLA B8 and circulating IgA anti-basement membrane zone antibody were more common in chronic bullous disease of childhood than adult linear IgA disease. There were no absolute differences among the three groups, and we suggest that adult linear IgA disease, chronic bullous disease of childhood, and childhood cicatricial pemphigoid are the same disease, with childhood cicatricial pemphigoid being a more severe form of chronic bullous disease of childhood.
Two patients aged 8 and 34 years, with linear IgA dermatosis and systemic symptoms are described. Both presented with fever and sore throat 5 to 10 days before the onset of their rash and both developed severe arthralgia accompanying the skin eruption. The joint pains resolved when the skin eruption was treated.
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The ultrastructural localization of the IgA deposits in the skin of 15 patients with linear IgA bullous dermatosis of adults (LAD), 13 with chronic bullous dermatosis of childhood (CBDC) and three with childhood cicatricial pemphigoid (CCP) were studied. The site of the antigen was determined using sera from two LAD, 13 CBDC and two CCP patients. In all 31 patients the IgA was located predominantly below the lamina lucida (sublamina densa). Similarly, the indirect immunoelectron microscopic studies demonstrated the antigen to be present at the same site, below the lamina densa. This suggests that in linear IgA bullous dermatosis the antibody reacts with the antigen in the sublamina densa region of the basement membrane zone.
A comparison of liver ultrasound and liver biopsy histology was made on 54 paired investigations from 28 patients about to receive or already receiving methotrexate for severe psoriasis. Ultrasound scans were reported as normal, or abnormal showing either fatty change or fibrosis. Eighteen of the scans were reported as normal and in no instance did the simultaneously-obtained liver biopsy show significant fibrosis. Thirty-one of the scans were reported as showing fatty change without fibrosis; in 12 cases the liver histology showed mild but significant fibrosis and in four cases fibrosis was of sufficient severity to necessitate methotrexate withdrawal. Five of the scans were reported as showing fibrosis and in all the biopsy showed significant fibrosis. This was of sufficient degree to necessitate methotrexate withdrawal in two cases. No patient with a normal ultrasound scan showed significant fibrosis and thus we concluded that such patients may be spared liver biopsy and safely continue with methotrexate therapy. Ultrasound cannot reliably distinguish between fatty change and fibrosis, so all patients with abnormal scans require liver biopsy.
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Thirty seven breast fed infants with eczema were studied to see whether changes in their mothers' diets affected their skin condition. Nineteen mothers and babies took part in a double blind crossover trial of exclusion of egg and cows' milk, and 18 took part in open exclusion of 11 foods followed by double blind challenge to those mothers whose infants seemed to respond. Babies were examined at the beginning and end of each dietary period, and the extent and severity of the rash were given a numerical score. The eczema improved in six infants when their mothers avoided egg and cows' milk and worsened again when these were reintroduced. Two infants suffered gastrointestinal reactions after maternal ingestion of egg and cows' milk, one developing colitis. Maternal dietary exclusion seems to benefit some breast fed babies with eczema.
Fifty-three patients with atopic eczema took part in a double blind controlled cross-over trial of an egg and cow's milk exclusion diet. Response to the diet was assessed in terms of areas affected, day and night time itch, and topical steroid usage. Twenty-five percent of the patients failed to comply adequately with the trial regime and were excluded from the analysis. Of the remaining 40 patients, ten appeared to benefit from the diet and were advised to continue egg and milk avoidance. This response rate to the diet was not statistically significant.
Forty nine eczematous infants who were still solely and exclusively breast fed and who had never received anything but breast milk were studied for evidence of sensitisation to foods. Thirty four similar infants without eczema formed a control group. The eczematous infants were divided into three groups according to clinical criteria: (1) definite atopic eczema; (2) possible atopic eczema; (3) atopic eczema unlikely. Twenty three infants showed cutaneous hypersensitivity to foods, usually egg and cows' milk. Seven of 14 infants in group 1 and nine of 20 in group 2 were sensitised compared with four of 15 in group 3 and three of 34 controls (p less than 0.01). Ovalbumin was detected in breast milk from 14 of 19 mothers tested after ingestion of egg, the concentrations being the same for mothers feeding eczematous and normal infants. Breast fed babies developing eczema may be sensitised by foods eaten by their mothers.
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An exclusively breast fed infant developed eczema and severe gastrointestinal symptoms when his mother took cow's milk or goat's milk, and eczema alone when she took soya milk. The cow's milk- and soya milk-induced symptoms were reproduced by double blind challenge.
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