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Dermatitis herpetiformis: a cutaneous manifestation of coeliac disease.
Ann Med. 2017 02; 49(1):23-31.AM

Abstract

Dermatitis herpetiformis (DH) is an itchy blistering skin disease with predilection sites on elbows, knees, and buttocks. Diagnosis is confirmed by showing granular immunoglobulin A deposits in perilesional skin. DH is one manifestation of coeliac disease; the skin symptoms heal with gluten free diet (GFD) and relapse on gluten challenge. Of the first-degree relatives, 5% may be affected by either condition. Tissue transglutaminase (TG2) is the autoantigen in coeliac disease and epidermal transglutaminase (TG3) in DH. Both diseases conditions exhibit TG2-specific autoantibodies in serum and small bowel mucosa; patients with DH have IgA-TG3 in the skin. There are some divergencies between these two phenotypes. One-fourth of DH patients do not have small bowel mucosal villous atrophy, but virtually all have coeliac-type inflammatory changes. The skin symptoms respond slowly to GFD. The incidence of coeliac disease is increasing, whereas the opposite is true for DH. A female predominance is evident in coeliac disease, while DH may be more common in males. Coeliac disease carries the risk of small intestinal T-cell lymphoma; in DH B-cell lymphomas at any site may prevail. Adult coeliac disease carries a slightly increased elevated mortality risk, whereas in DH, the relative mortality rate is significantly decreased. Key messages Dermatitis herpetiformis is a cutaneous manifestation of coeliac disease; both conditions are genetically determined and gluten-dependent. Gastrointestinal symptoms and the degree of villous atrophy are less obvious in dermatitis herpetiformis than in coeliac disease. Both show tissue transglutaminase (TG2) specific autoantibodies in serum and small bowel mucosa. In addition, TG3-targeted IgA antibodies are found in the skin of DH patients Both conditions carry an increased elevated risk of lymphoma, in coeliac disease small intestinal T-cell lymphoma, in dermatitis herpetiformis mainly B-cell lymphoma at various sites. Coeliac disease is currently eight times more common that DH; the incidence of DH is decreasing in contrast to that of coeliac disease, where it is increasing.

Authors+Show Affiliations

a Department of Gastroenterology and Alimentary Tract Surgery , Tampere University Hospital , Tampere , Finland.b Department of Dermatology , Tampere University Hospital , Tampere , Finland. c School of Medicine , University of Tampere , Tampere , Finland.b Department of Dermatology , Tampere University Hospital , Tampere , Finland. c School of Medicine , University of Tampere , Tampere , Finland.c School of Medicine , University of Tampere , Tampere , Finland. d Department of Internal Medicine , Tampere University Hospital , Tampere , Finland.b Department of Dermatology , Tampere University Hospital , Tampere , Finland. c School of Medicine , University of Tampere , Tampere , Finland.

Pub Type(s)

Journal Article
Review
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

27499257

Citation

Collin, Pekka, et al. "Dermatitis Herpetiformis: a Cutaneous Manifestation of Coeliac Disease." Annals of Medicine, vol. 49, no. 1, 2017, pp. 23-31.
Collin P, Salmi TT, Hervonen K, et al. Dermatitis herpetiformis: a cutaneous manifestation of coeliac disease. Ann Med. 2017;49(1):23-31.
Collin, P., Salmi, T. T., Hervonen, K., Kaukinen, K., & Reunala, T. (2017). Dermatitis herpetiformis: a cutaneous manifestation of coeliac disease. Annals of Medicine, 49(1), 23-31. https://doi.org/10.1080/07853890.2016.1222450
Collin P, et al. Dermatitis Herpetiformis: a Cutaneous Manifestation of Coeliac Disease. Ann Med. 2017;49(1):23-31. PubMed PMID: 27499257.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Dermatitis herpetiformis: a cutaneous manifestation of coeliac disease. AU - Collin,Pekka, AU - Salmi,Teea T, AU - Hervonen,Kaisa, AU - Kaukinen,Katri, AU - Reunala,Timo, Y1 - 2016/12/14/ PY - 2016/8/9/pubmed PY - 2017/11/29/medline PY - 2016/8/9/entrez KW - Bullous dermatoses KW - coeliac disease KW - dermatitis herpetiformis KW - gluten intolerance KW - tissue transglutaminase SP - 23 EP - 31 JF - Annals of medicine JO - Ann Med VL - 49 IS - 1 N2 - Dermatitis herpetiformis (DH) is an itchy blistering skin disease with predilection sites on elbows, knees, and buttocks. Diagnosis is confirmed by showing granular immunoglobulin A deposits in perilesional skin. DH is one manifestation of coeliac disease; the skin symptoms heal with gluten free diet (GFD) and relapse on gluten challenge. Of the first-degree relatives, 5% may be affected by either condition. Tissue transglutaminase (TG2) is the autoantigen in coeliac disease and epidermal transglutaminase (TG3) in DH. Both diseases conditions exhibit TG2-specific autoantibodies in serum and small bowel mucosa; patients with DH have IgA-TG3 in the skin. There are some divergencies between these two phenotypes. One-fourth of DH patients do not have small bowel mucosal villous atrophy, but virtually all have coeliac-type inflammatory changes. The skin symptoms respond slowly to GFD. The incidence of coeliac disease is increasing, whereas the opposite is true for DH. A female predominance is evident in coeliac disease, while DH may be more common in males. Coeliac disease carries the risk of small intestinal T-cell lymphoma; in DH B-cell lymphomas at any site may prevail. Adult coeliac disease carries a slightly increased elevated mortality risk, whereas in DH, the relative mortality rate is significantly decreased. Key messages Dermatitis herpetiformis is a cutaneous manifestation of coeliac disease; both conditions are genetically determined and gluten-dependent. Gastrointestinal symptoms and the degree of villous atrophy are less obvious in dermatitis herpetiformis than in coeliac disease. Both show tissue transglutaminase (TG2) specific autoantibodies in serum and small bowel mucosa. In addition, TG3-targeted IgA antibodies are found in the skin of DH patients Both conditions carry an increased elevated risk of lymphoma, in coeliac disease small intestinal T-cell lymphoma, in dermatitis herpetiformis mainly B-cell lymphoma at various sites. Coeliac disease is currently eight times more common that DH; the incidence of DH is decreasing in contrast to that of coeliac disease, where it is increasing. SN - 1365-2060 UR - https://www.unboundmedicine.com/medline/citation/27499257/Dermatitis_herpetiformis:_a_cutaneous_manifestation_of_coeliac_disease_ L2 - https://www.tandfonline.com/doi/full/10.1080/07853890.2016.1222450 DB - PRIME DP - Unbound Medicine ER -