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-insoluble proteins found in many cereals. Autoantibodies against gliadin and...
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References
Nicolas ME et al. (2003) Dermatitis herpetiformis. Int J Dermatol 42:588–600
Dieterich et al. (1997) Identification of tissue transglutaminase as the autoantigen of celiac disease. Nat Med 3:797–801
Sardy et al. (2002) Epidermal transglutaminase (TGase 3) is the autoantigen of dermatitis herpetiformis. J Ex Med 195:747–757
Donaldson MR et al. (2007) Epidermal transglutaminase deposits in perilesional and uninvolved skin in patients with dermatitis herpetiformis. J Invest Dermatol 127:1268–1271
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Hofmann, S., Bruckner-Tuderman, L. (2009). Dermatitis Herpetiformis. In: Lang, F. (eds) Encyclopedia of Molecular Mechanisms of Disease. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-29676-8_471
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DOI: https://doi.org/10.1007/978-3-540-29676-8_471
Publisher Name: Springer, Berlin, Heidelberg
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