Dermatitis herpetiformis Duhring

Der­ma­ti­tis her­pe­ti­for­mis Duh­ring is a chro­nic, blis­te­ring auto­im­mu­ne dise­a­se of the skin that is clo­se­ly asso­cia­ted with coeli­ac dise­a­se. It affects around 10 to 20 per­cent of peo­p­le with glu­ten into­le­rance and usual­ly occurs bet­ween the ages of 30 and 40, with men being affec­ted more fre­quent­ly than women. The dise­a­se is cau­sed by an immu­ne reac­tion against glu­ten, a pro­te­in found in wheat, rye and bar­ley. The body pro­du­ces anti­bo­dies which are depo­si­ted in the skin and trig­ger an inflamm­a­to­ry reac­tion. The dise­a­se is cha­rac­te­ri­zed by very itchy, bur­ning blis­ters and papu­les that are arran­ged sym­me­tri­cal­ly. Pre­fer­red sites are the exten­sor sides of the elbows and kne­es, buttocks, should­ers, neck and scalp. The itching is often so seve­re that the blis­ters are scrat­ched open befo­re they are real­ly visi­ble. This results in crusts and scratch marks. The sym­ptoms occur in epi­so­des and can per­sist for years. Dia­gno­sis is made by taking a skin bio­psy from healt­hy skin next to an affec­ted area. Direct immu­no­fluo­re­s­cence shows typi­cal depo­sits of IgA anti­bo­dies on the base­ment mem­bra­ne. In addi­ti­on, blood tests for spe­ci­fic anti­bo­dies and a bio­psy of the small intesti­ne are car­ri­ed out to detect coeli­ac dise­a­se. The most important tre­at­ment is a lifel­ong, strict­ly glu­ten-free diet. This usual­ly leads to a signi­fi­cant impro­ve­ment in the skin sym­ptoms after months and also pre­vents the intesti­nal dama­ge cau­sed by coeli­ac dise­a­se. The drug dap­so­ne, which effec­tively sup­pres­ses itching and blis­te­ring, is used for rapid sym­ptom reli­ef. The pro­gno­sis is good with a con­sis­tent glu­ten-free diet. Wit­hout tre­at­ment, the­re is an increased risk of lym­pho­ma and other com­pli­ca­ti­ons of coeli­ac disease.