Epidemiology

Dermatitis herpetiformis is a rare disease, with a prevalence between 10 and 75 cases per 100,000 population and an incidence ranging from less than 1.0 to 3.5 cases per 100,000 inhabitants.[5] There is no clear sex predominance, although some studies report a slight male predominance.[5][6][7]

Dermatitis herpetiformis usually occurs during adulthood, with a higher incidence in the fourth and fifth decade.[5] However, several paediatric cases have been reported.[4]

White people are more affected than other ethnicities, with northern Europe (particularly Finland) and North America being the areas with highest incidence and prevalence.[5][8] By contrast, dermatitis herpetiformis is extremely rare in Africans, African-Americans, and Asians. Japanese people may present with a variant, the so-called fibrillar-type dermatitis herpetiformis, that shows distinct features and seems not to be associated with coeliac disease.[9]

Contemporary data suggest that the incidence and prevalence of dermatitis herpetiformis are reducing; by contrast, for coeliac disease, they are increasing.[5][6] The reason for the reduction in incidence and prevalence of dermatitis herpetiformis has not been confirmed, but may be related to earlier diagnosis and an early introduction of gluten-free diet, which may prevent dermatitis herpetiformis from developing in some patients with coeliac disease.

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