Prognosis

Disease course

Dermatitis herpetiformis, if not treated, runs a chronic relapsing course with exacerbations followed by phases in which signs and symptoms are less severe.[1] After a gluten-free diet is adopted, most patients slowly improve until they achieve complete remission. However, cutaneous manifestations may last for months to years after starting a gluten-free diet.[62] In these patients, treatment with dapsone is often able to induce remission in just a few days, although the treatment should be prolonged until all skin lesions have resolved as early discontinuation may cause relapse.[63]

Reintroduction of gluten

Dermatitis herpetiformis is nearly always associated with celiac disease and a lifelong gluten-free diet should be maintained.[64] Almost all patients with long-lasting remission will experience a relapse if they reintroduce gluten into their diet. In one study, 10 out of 86 patients with dermatitis herpetiformis did not show relapses of cutaneous manifestations, although their intestinal status was not assessed.[50] More recently, 20 patients with dermatitis herpetiformis reintroduced gluten into their diet after complete remission of the disease. At 1 year, all but one showed skin or intestinal relapse.[63]

Mortality

The life expectancy of people with dermatitis herpetiformis appears to be increased compared with that of the general population and that of people with celiac disease.[65][66] The increased life expectancy rates compared with the general population may be explained by a number of reasons, including strict adherence to a gluten-free diet, and lower levels of smoking and hypercholesterolemia in people with dermatitis herpetiformis.[65] Complications that may occur in patients with celiac disease and may lead to death, such as lymphoma, although reported, are very rare in patients with dermatitis herpetiformis and the risk may be minimized by following a gluten-free diet.[67]

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