Histology: Early lesions of dermatitis herpetiformis show subepidermal clefting with a neutrophil-rich infiltrate in the papillary dermis. As the lesions progress, subepidermal blistering becomes prominent, and the papillary dermis is filled with neutrophils. The histological findings of dermatitis herpetiformis can be difficult to differentiate from those of linear IgA bullous dermatosis on routine hematoxylin and eosin staining. Direct immunofluorescence is required to differentiate the two diseases. The direct immunofluorescence staining pattern in dermatitis herpetiformis is that of a speckled arrangement of IgA within the papillary dermis. In linear IgA bullous disease, as the name implies, a linear pattern along the basement membrane zone is seen.
Treatment: The treatment of dermatitis herpetiformis is twofold. The first aspect of therapy is to control the itching and blistering. This can be rapidly achieved with dapsone or sulfapyridine. The response to these two medications is remarkably quick, with most patients noticing near-resolution of their symptoms within 1 day. In cases of suspected dermatitis herpetiformis that has not been confirmed histologically, dapsone can be used as a therapeutic test: If the patient sees a rapid response after the first day of dapsone therapy, the diagnosis is most certainly dermatitis herpetiformis. Dapsone or alternative medications can treat the blistering and pruritus, but they do not decrease the long-term risk of small-bowel lymphoma. The only means of decreasing and removing the risk of lymphoma is to have the patient adhere to a strict gluten-free diet. This requires nutritional education. If patients are able to entirely avoid gluten-containing products, not only will the rash resolve, but the gastrointestinal abnormalities will resolve, and the risk of lymphoma will return to that of the general population.
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