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Dermatitis Herpetiformis Duhring

  • Chapter
Autoimmune Diseases of the Skin

Summary

Dermatits herpetiformis (DH) is a pruritic autoimmune subepidermal bullous disease and is considered as a specific cutaneous manifestation of celiac disease (CD). The diagnosis of DH is based on the detection of granular IgA deposits in biopsies of normal-appearing perilesional skin. CD and DH patients have the same genetic predisposition and demonstrate IgA anti-endomysium antibodies in their sera. These antibodies are directed against tissue transglutaminase. Serum levels of IgA antibodies to tissue transglutaminase reflect the extent of histopathologic changes of the small bowel and are routinely assessed in patients with DH and CD. The autoantigen of DH is epidermal transglutaminase which is found in the skin of DH-patients at the same site as the granular IgA deposits. Detection of anti-IgA antibodies against epidermal transglutaminase is a sensitive serological test in the diagnosis of DH. Skin lesions can be controlled by dapsone, but the drug does not affect the small bowel disease. For CD, the treatment of choice is a gluten-free diet that should be maintained for a lifetime. In most patients, the diet eventually results in remission of the skin lesions, a clearance of IgA deposits from the skin and an improvement of the bowel disease. The question why some patients with mild CD produce antibodies against epidermal transglutaminase and develop DH still remains to be elucidated.

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  1. Department of Dermatology, University of Lübeck, Ratzeburger Allee 160, D-23538, Lübeck, Germany

    Christian Rose MD & Detlef Zillikens

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Michael Hertl

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Rose, C., Zillikens, D. (2011). Dermatitis Herpetiformis Duhring. In: Hertl, M. (eds) Autoimmune Diseases of the Skin. Springer, Vienna. https://doi.org/10.1007/978-3-211-99225-8_5

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