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Dermatitis herpetiformis: pathognomonic transglutaminase IgA deposits in the skin and excellent prognosis on a gluten-free diet.

Abstract

Dermatitis herpetiformis (DH) is an itchy, blistering skin disease with sites of predilection at the elbows, knees and buttocks. Although DH is mostly asymptomatic, all patients exhibit small bowel villous atrophy or at least coeliac-type inflammatory changes. Deposition of immunoglobulin A (IgA) in the papillary dermis is a key diagnostic feature of DH. Epidermal transglutaminase (TG3) is the antigen for IgA deposited in the skin, and tissue transglutaminase (TG2) is the antigen for IgA deposited in the small bowel mucosa. Clinically silent, but immunologically active coeliac disease in the gut appears to result in IgA TG3 antibody complexes aggregated into DH skin. The prevalence of DH in northern Europe is high (30-75/100,000), but its incidence is decreasing, possibly due to increased recognition of subclinical coeliac disease. The rash and small bowel heal on a gluten-free diet, which is a life-long treatment. The risk of non-Hodgkin's lymphoma is increased, but in patients with DH who adhere strictly to a gluten-free diet long-term prognosis is excellent.

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  • Authors+Show Affiliations

    ,

    Department of Dermatology, Tampere University Hospital PO Box 2000, FIN-33521 Tampere, Finland. timo.reunala@uta.fi.

    ,

    Source

    Acta dermato-venereologica 95:8 2015 Nov pg 917-22

    MeSH

    Celiac Disease
    Clinical Enzyme Tests
    Dermatitis Herpetiformis
    Diet, Gluten-Free
    GTP-Binding Proteins
    Humans
    Immunoglobulin A
    Intestinal Mucosa
    Intestine, Small
    Transglutaminases

    Pub Type(s)

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

    Language

    eng

    PubMed ID

    26059085

    Citation

    Reunala, Timo, et al. "Dermatitis Herpetiformis: Pathognomonic Transglutaminase IgA Deposits in the Skin and Excellent Prognosis On a Gluten-free Diet." Acta Dermato-venereologica, vol. 95, no. 8, 2015, pp. 917-22.
    Reunala T, Salmi TT, Hervonen K. Dermatitis herpetiformis: pathognomonic transglutaminase IgA deposits in the skin and excellent prognosis on a gluten-free diet. Acta Derm Venereol. 2015;95(8):917-22.
    Reunala, T., Salmi, T. T., & Hervonen, K. (2015). Dermatitis herpetiformis: pathognomonic transglutaminase IgA deposits in the skin and excellent prognosis on a gluten-free diet. Acta Dermato-venereologica, 95(8), pp. 917-22. doi:10.2340/00015555-2162.
    Reunala T, Salmi TT, Hervonen K. Dermatitis Herpetiformis: Pathognomonic Transglutaminase IgA Deposits in the Skin and Excellent Prognosis On a Gluten-free Diet. Acta Derm Venereol. 2015;95(8):917-22. PubMed PMID: 26059085.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Dermatitis herpetiformis: pathognomonic transglutaminase IgA deposits in the skin and excellent prognosis on a gluten-free diet. AU - Reunala,Timo, AU - Salmi,Teea T, AU - Hervonen,Kaisa, PY - 2015/6/11/entrez PY - 2015/6/11/pubmed PY - 2016/8/17/medline SP - 917 EP - 22 JF - Acta dermato-venereologica JO - Acta Derm. Venereol. VL - 95 IS - 8 N2 - Dermatitis herpetiformis (DH) is an itchy, blistering skin disease with sites of predilection at the elbows, knees and buttocks. Although DH is mostly asymptomatic, all patients exhibit small bowel villous atrophy or at least coeliac-type inflammatory changes. Deposition of immunoglobulin A (IgA) in the papillary dermis is a key diagnostic feature of DH. Epidermal transglutaminase (TG3) is the antigen for IgA deposited in the skin, and tissue transglutaminase (TG2) is the antigen for IgA deposited in the small bowel mucosa. Clinically silent, but immunologically active coeliac disease in the gut appears to result in IgA TG3 antibody complexes aggregated into DH skin. The prevalence of DH in northern Europe is high (30-75/100,000), but its incidence is decreasing, possibly due to increased recognition of subclinical coeliac disease. The rash and small bowel heal on a gluten-free diet, which is a life-long treatment. The risk of non-Hodgkin's lymphoma is increased, but in patients with DH who adhere strictly to a gluten-free diet long-term prognosis is excellent. SN - 1651-2057 UR - https://www.unboundmedicine.com/medline/citation/26059085/full_citation L2 - https://www.medicaljournals.se/acta/content/abstract/10.2340/00015555-2162 DB - PRIME DP - Unbound Medicine ER -