Reference Work Entry

Encyclopedia of Molecular Mechanisms of Disease

pp 516-516

Dermatitis Herpetiformis

  • Silke HofmannAffiliated withInstitute of Physiology, University of TuebingenDepartment of Dermatology, University Medical Center
  • , Leena Bruckner-TudermanAffiliated withInstitute of Physiology, University of TuebingenDepartment of Dermatology, University Medical Center

Synonyms

Duhring’s disease

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 [1].

Prevalence

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.

Genes

Association with HLA class II genes HLA-DQA1*0501, DQB1*02, and to a lesser extent with the HLA-DQA1*03, DQB1*0302.

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-insolubl ...

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