Synonyms
Dermatitis herpetiformis Duhring; Duhring’s disease; Duhring-Brocq disease; Morbus Duhring
Definition
Dermatitis herpetiformis is a rare, autoimmune bullous dermatosis associated with gluten-sensitive enteropathy and characterized by granular deposition of IgA in the dermal papillae. IgA antibodies directed against transglutaminases lead to activation of the complement system, resulting in chemotaxis of neutrophils into the papillary dermis. The release of enzymes from neutrophils causes an alteration or destruction of basement membrane components leading to blister formation. The typical lesions are grouped papulovesicules, which are usually excoriated due to intense pruritus.
An associated gluten-sensitive enteropathy can be found in approximately 90 % of patients upon histological examination of endoscopic biopsies. However, only about 20 % show clinical symptoms, which are usually mild. Approximately 20 % of patients suffering from coeliac disease develop dermatitis...
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Sárdy, M., Kárpáti, S., Merkl, B., Paulsson, M., & Smyth, N. (2002). Epidermal transglutaminase (TGase 3) is the autoantigen of dermatitis herpetiformis. The Journal of Experimental Medicine, 195(6), 747–757.
Smith, J. B., Tulloch, J. E., Meyer, L. J., & Zone, J. J. (1992). The incidence and prevalence of dermatitis herpetiformis in Utah. Archives of Dermatology, 128(12), 1608–1610.
Viljamaa, M., Kaukinen, K., Pukkala, E., Hervonen, K., Reunala, T., & Collin, P. (2006). Malignancies and mortality in patients with coeliac disease and dermatitis herpetiformis: 30-year population-based study. Digestive and Liver Disease, 38(6), 374–380.
Warren, S. J. P., & Cockerell, C. J. (2002). Characterization of a subgroup of patients with dermatitis herpetiformis with nonclassical histologic features. American Journal of Dermatopathology, 24(4), 305–308.
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© 2016 Springer International Publishing Switzerland
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Pfaltz, K. (2016). Dermatitis Herpetiformis. In: Massi, D. (eds) Dermatopathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-30006-1_410
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DOI: https://doi.org/10.1007/978-3-319-30006-1_410
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Publisher Name: Springer, Cham
Print ISBN: 978-3-319-30005-4
Online ISBN: 978-3-319-30006-1
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