Dermatitis herpetiformis

Abstract

Summary Dermatitis herpetiformis (DH) is a cutaneous manifestation of coeliac disease (CD), which causes an itching and blistering rash, typically on the elbows, knees and buttocks. DH and CD share a similar genetic background, small bowel mucosal alterations, and an autoimmune response against tissue transglutaminase in the serum and small bowel. DH is typically diagnosed during adulthood, and it is slightly more common among males than females. The incidence of DH seems to be decreasing, in contrast to the detected four‐fold increase in the incidence of CD. In addition to typical clinical picture, diagnosis of DH relies on the demonstration by direct immunofluorescence of pathognomonic granular IgA deposits in the papillary dermis. Circulating tissue transglutaminase antibodies support the diagnosis, but their absence does not exclude DH. Obtainment of small bowel mucosal biopsies is not necessary when DH is diagnosed, but if performed, the majority of patients are found to have villous atrophy, and even those with normal villous architecture evince CD‐type inflammation. The treatment of choice in DH is a strict, life‐long adherence to a gluten‐free diet (GFD). In addition to alleviating the symptoms of DH and healing the small bowel mucosal damage, a GFD increases the quality of life for patients, and decreases the risk for lymphoma in DH. Further, the mortality rate of patients with DH treated with a GFD seems to be lower than that of the general population. However, as changing to a GFD has a rather slow effect on the DH rash, patients with severe skin symptoms should additionally be treated with dapsone medication. This review article is based on a presentation given at the British Society for Medical Dermatology blistering skin diseases meeting 2019.

Autoantibodies Against the Immunodominant Bullous Pemphigoid Epitopes Are Rare in Patients With Dermatitis Herpetiformis and Coeliac Disease

“…Proteolytic gliadin peptides from ingested gluten induce production of IgA class autoantibodies against both native and gliadin-associated tissue transglutaminase 2 (TG2) (2). These antibodies are detectable in the serum and small bowel mucosa of CD patients, but in only about 75% of DH patients (6,7). Typically the sera of DH patients contain anti-epidermal transglutaminase (TG3) IgA autoantibodies, which show up on direct immunofluorescence microscopy (DIF) analysis of the skin as granular IgA deposits in the papillary dermis (8,9).…”

“…TG3 ELISA tests are also available, but are currently used only for research purposes (2). A lifelong gluten-free diet is the basis of treatment of both CD and DH (6).…”

Subcorneal Pustular Dermatosis with Temporary Autoimmune Features during COVID-19 Pandemic

“…The hypopyon is seen mainly in Sneddon-Wilkinson disease and Duhring disease [5]. In Duhring disease, however, autoantibodies to gliadin and transglutaminase are present and the cutaneous lesions are extremely pruritic [6].…”

Transglutaminase 3: The Involvement in Epithelial Differentiation and Cancer

“…Thus, patients with DH have gastro-intestinal symptoms similar to patients with CD. Most frequently, they are diagnosed with villous atrophy and seldom only minor enteropathy [ 131 ]. At the molecular level, in the course of CD, TGM2 deamidates gliadin proteins contained in gluten, which leads to the formation of immunogenic epitopes [ 132 ].…”