Dermatitis herpetiformis: a cutaneous manifestation of coeliac disease.

Dermatitis herpetiformis: a cutaneous manifestation of coeliac disease. Ann Med. 2016 Aug 8;:1-25 Authors: Collin P, Salmi TT, Hervonen K, Kaukinen K, Reunala T 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 relapses 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 sligthly increased elevated mortality risk, whereas in DH the relativ...
Source: Annals of Medicine - Category: Internal Medicine Tags: Ann Med Source Type: research