Klinik, Histologie und Immunpathologie bei 32 Patienten mit Dermatitis herpetiformis Duhring

Background: Dermatitis herpetiformis is a chronic severely pruritic dermatosis. It is a cutaneous manifestation of celiac disease. The aim of our study was to collect clinical, histological and immunopathological data on patients who were treated in the University Departments of Dermatology in Würzburg and Lübeck from 1996 to 2008. Patients and Methods: We retrospectively analyzed 32 patients. Only patients with positive findings on direct immunofluorescence microscopy were included in this study. Results: All patients demonstrated skin lesions in the predilection areas of knees, elbows, gluteal region and scalp. The male to female ratio was 1.5 : 1 and the average age was 43 years. The interval between the first symptoms and diagnosis ranged from 6 weeks to 20 years. Direct immunofluorescence microscopy showed that granular IgA deposits were more often found continuously along the dermal-epidermal junction rather than focally in the tips of the dermal papillae. Results of small intestinal biopsies were available from 29 patients and confirmed the presence of celiac disease in all cases. None of the patients reported gastrointestinal symptoms. IgA antibodies against tissue transglutaminase and epidermal transglutaminase were found in 88% and 94% of patient sera, respectively. Conclusions: The detection of IgA autoantibodies against epidermal transglut-aminase is the most sensitive serological test in the diagnosis of dermatitis herpetiformis. Our observations confirm that patients with dermatitis herpetiformis usually do not demonstrate apparent gastrointestinal symptoms.