Dermatitis Herpetiformis

Abstract

Dermatitis herpetiformis (DH) is a chronic, polymorphic, blistering, and pruritic skin disease that develops mostly in patients with a latent gluten-sensitive enteropathy (GSE) called celiac disease. DH patients usually present with skin manifestations only and are not aware of the underlying gluten sensitivity. Perilesional granular IgA deposition at the uppermost papillary layer and lesional neutrophilic accumulation with subepidermal blister formation together prove the diagnosis. Almost all DH patients have circulating gluten-induced transglutaminase 2 (TG2) or endomysium antibodies characteristic for GSE, but they also have anti-TG3 IgA antibodies present only in some celiac patients. Associated autoimmunities, endocrinological, neurological, gastrointestinal pathologies need a careful multidisciplinary follow-up. Family screening for gluten sensitivity by IgA TG2 antibodies or exceptionally by the characteristic HLA background verification is suggested.