PubMed · 12988610
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H M M WENTHOLT, E JANSEN. 1952. Some observations on pemphigus vegetans.. https://doi.org/10.1159/000256891
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A 54-year old man with atypical dermatitis herpetiformis Duhring lasting about three years is reported. The lesions were located exclusively in the pubic area, groins, inner thighs and perianal regions. Routine histopathology and the direct as well as the indirect immunofluorescence supported the diagnosis. Application of a gluten-free diet and dapsone alleviated the symptoms.
Dermatitis herpetiformis is an autoimmune blistering disease characterized by granular deposits of immunoglobulin A (IgA) in dermal papillae. Erythema elevatum diutinum is a chronic form of vasculitis associated with IgA paraproteinemia. We report a patient with dermatitis herpetiformis who developed characteristic erythema elevatum diutinum lesions on the elbows and knees despite sulfone therapy. We speculate that the different patterns of IgA deposition in the skin account for the clinical manifestation of these 2 uncommon disorders.