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Dermatitis herpetiformis

Author
Christopher Hull, MD
Section Editor
John J Zone, MD
Deputy Editor
Abena O Ofori, MD

INTRODUCTION

Dermatitis herpetiformis (DH) is an uncommon autoimmune cutaneous eruption associated with gluten sensitivity. Affected patients typically develop intensely pruritic inflammatory papules and vesicles on the forearms, knees, scalp, or buttocks (picture 1A-G). The vast majority of patients with DH also have an associated gluten-sensitive enteropathy (celiac disease). In most of these patients, the enteropathy is asymptomatic.

DH usually responds well to treatment. Dapsone and a gluten-free diet are the primary interventions for the management of this disease.

The pathogenesis, diagnosis, and treatment of DH will be discussed here. Celiac disease is reviewed separately. (See "Pathogenesis, epidemiology, and clinical manifestations of celiac disease in adults" and "Diagnosis of celiac disease in adults" and "Epidemiology, pathogenesis, and clinical manifestations of celiac disease in children".)

EPIDEMIOLOGY

DH is an uncommon disorder that most frequently occurs in individuals of northern European heritage. Epidemiologic studies from northern Europe have found incidence rates between 0.4 and 3.5 per 100,000 people per year and prevalence rates between 1.2 and 75.3 per 100,000 people [1-8]. A population-based study in Utah, a location with a relatively high proportion of individuals with northern European ancestry, found incidence and prevalence rates similar to those reported in Europe [9]. Between 1978 and 1987, the incidence of DH was 0.98 per 100,000 people per year, and the prevalence of DH in 1987 was 11.2 per 100,000 people.

The incidence of DH may be decreasing. An analysis of patient data from the Clinical Practice Research Datalink in the United Kingdom found a decline in incidence from 1.82 per 100,000 person-years in 1990 to 0.8 per 100,000 person-years in 2011 [10]. A Finnish study that compared the incidence of DH diagnosed within a university hospital district in three successive decades, beginning in 1970, also found decreasing rates of DH [3]. The estimated annual incidence for each decade was 5.2, 2.9, and 2.7 per 100,000 people, respectively. The reason for the observed decline in DH incidence has not been confirmed. Increased detection and treatment of mild celiac disease is a potential contributor. (See 'Pathogenesis' below.)

                           

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Literature review current through: Nov 2016. | This topic last updated: Fri Oct 14 00:00:00 GMT+00:00 2016.
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