Definition and Characteristics
Autoimmune blistering disease of the skin, with granular IgA deposits in the papillary dermis and circulating autoantibodies to transglutaminase(s). Association with (subclinical) celiac disease (gluten-sensitive enteropathy). Dermatological hallmarks are intensely itching small papules, vesicles and erosions, often symmetrically distributed on the extensor surfaces of the extremities .
Dermatitis herpetiformis is more common in Northern countries. The prevalence is not known, but figures of 1:1,000-1:100,000 have been reported.
Molecular and Systemic Pathophysiology
Both celiac disease and dermatitis herpetiformis share identical jejunal pathology and can be induced by ingestion of gluten, a mass of water-insoluble proteins found in many cereals. Autoantibodies