Dominant dystrophic epidermolysis bullosa associated with pyloric stenosis and congenital absence of skin.
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Biomedical subjects
Publications and source records attributed to B Lagrange.
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INTRODUCTION: Pustular vasculitis is a classical manifestation of Behçet's disease. We report a case where the papulopustular eruption formed an arch on the skin and the histology examination showed predominant lymphocytic infiltration. CASE REPORT: A 29 year-old with a past history of recurrent buccal aphthosis had developed episodic papulopustular eruptions over the past 3 years. The diagnostic of Behçet's disease was made on the basis of an association with lymphocytic meningis, uveitis and erythema nodosum. General corticosteroid therapy was effective, but did not prevent skin relapse, controlled with colchicine. DISCUSSION: Pustular vasculitis usually presents with pustular eruptions on purpuric lesions and polynuclear infiltration of the dermis. Predominant lymphocytic infiltration is usually due to a pathergy phenomenon and would be specific for Behçet's disease. In our patient, colchicine was effective, although the mechanism remains to be explained.
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IF: negative test: less than 10 UI/ml; doubtful test 10-100 UI/ml; positive test greater than 100 UI/ml. HA: negative test: less than 8 dilutions; doubtful test 16-24; positive test greater than 128. The antibodies kinetics vary with the pattern of the infection (primary infection or reinfection), with the method of identification, and with the subjects. In the case of primary infection, the IgM appear since the first week following the acquisition of the infection; they reach their highest titre at the first month (variability: 15th day-3rd month); they disappear during the fourth month (variability: 2-9 months). The antibodies detected by HA after 2 ME must be reduced by at least 3 dilutions to be significant. The IgG appear later. They are detected at the beginning of the second week by IF or of the 4th month by HA, reach their highest titre at the 4th month by IF (variability: 2-9 months) or at the 9th month by HA (variability: 4th month-2nd year); they then decrease to reach lower titres within the space of 1 or 5 years. The antibodies detected in the case of reinfection are usually of higher titre with a steeper rate of increase, and are more persistent than following a primary infection.
The purpose of oral food challenges is to detect food intolerance. Challenges of food additives and drugs are not often used in children. The authors report methods and protocols that they used for food, food additives and contaminants and emphasize the potential dangers of these tests. Atopic diseases and chronic urticaria are the principal indications. Cow's milk, eggs and fish are the allergens that are most often incriminated. Oral challenges are only to be done as part of an integrated investigation together with skin tests, in vitro tests and a study of intestinal permeability. Food avoidance and therapy should be decided after the challenge data are available.