Abstract

Dermatitis herpetiformis (DH) is an autoimmune bullous disease characterized by intensely pruritic, chronic, and recurrent vesicles on extensor surfaces such as the elbows, knees, and buttocks. The collection of neutrophils at the papillary tips is the typical histopathological finding, and a characteristic diagnostic feature is granular immunoglobulin A deposition in the papillary dermis by direct immunofluorescence. DH is closely associated with gluten sensitive enteropathy and is considered a cutaneous manifestation of gluten sensitivity; i.e., an extra-intestinal presentation of celiac disease. Gluten free diet is the first-line therapy for patients with DH and dapsone is also effective. DH preferentially affects Caucasians who carry human leukocyte antigen (HLA)-DQ2 or HLA-DQ8. The major autoantigen is epidermal transglutaminase. This review focuses on the confirmedfeatures of DH and our recent findings specific to DH in Japanese patients.

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