What is dermatitis herpetiformis?

➢ Dermatitis herpetiformis is an autoimmune blistering disorder associated with gluten sensitivity that is characterized by extremely pruritic grouped vesicles and papules. ➢ The disease affects predominantly males, often in the third and fourth decades of life. ➢ Up to 80% of cases are associated with celiac disease; ➢ conversely, only a small fraction of patients with celiac disease develop dermatitis herpetiformis. ➢ Like celiac disease, dermatitis herpetiformis responds to a gluten-free diet. ➢ Genetically predisposed individuals develop IgA antibodies to dietary gluten as well as IgA autoantibodies that cross-react with endomysium and tissue transglutaminases, including epidermal transglutaminase expressed by keratinocytes. ➢ By direct immunofluorescence, the skin shows discontinuous, granular deposits of IgA selectively localized in the tips of dermal papillae