title: Dermatitis Herpetiformis Refractory to Gluten-free Dietary Treatment.
abstract: Dermatitis herpetiformis (DH) is a blistering skin disease, which is regarded as an extra-intestinal manifestation of coeliac disease. Refractory cases of coeliac disease, that do not respond to a gluten-free diet and which carry an increased risk of lymphoma, are well-known in coeliac disease. To determine whether refractory cases of DH with active rash and persistent small bowel atrophy occur we analysed our series of 403 patients with DH. Seven (1.7%) patients, who had been on a gluten-free diet for a mean of 16 years, but who still required dapsone to treat the symptoms of DH, were identified. Of these, one patient died from mucinous adenocarcinoma before re-examination. At re-examination skin immunoglobulin A (IgA) deposits were found in 5/6 refractory and 3/16 control DH patients with good dietary response. Small bowel mucosa was studied at re-examination from 5 refractory and 8 control DH patients and was normal in all 5 refractory and 7/8 control DH patients. One refractory DH patient died from adenocarcinoma, but no lymphoma developed in any of the patients. This study documents for the first time refractory DH, in which the rash is non-responsive to a gluten-free diet, but the small bowel mucosa heals. This differs from refractory coeliac disease, in which the small bowel mucosa does not heal on a gluten-free diet.
authors.affiliation: Department of Dermatology, Tampere University Hospital and University of Tampere, FIN-33240 Tampere, Finland. kaisa.hervonen@uta.fi.
authors.forename: Kaisa
authors.initials: K
authors.lastname: Hervonen
authors.forename: Teea T
authors.initials: TT
authors.lastname: Salmi
authors.forename: Tuire
authors.initials: T
authors.lastname: Ilus
authors.forename: Kaija
authors.initials: K
authors.lastname: Paasikivi
authors.forename: Martine
authors.initials: M
authors.lastname: Vornanen
authors.forename: Kaija
authors.initials: K
authors.lastname: Laurila
authors.forename: Katri
authors.initials: K
authors.lastname: Lindfors
authors.forename: Keijo
authors.initials: K
authors.lastname: Viiri
authors.forename: Päivi
authors.initials: P
authors.lastname: Saavalainen
authors.forename: Pekka
authors.initials: P
authors.lastname: Collin
authors.forename: Katri
authors.initials: K
authors.lastname: Kaukinen
authors.forename: Timo
authors.initials: T
authors.lastname: Reunala
mesh_terms: D000293:Adolescent; D000328:Adult; D001284:Atrophy; D001706:Biopsy; D002446:Celiac Disease / Q000175:diagnosis / Q000178:diet therapy* / Q000276:immunology; D002648:Child; D003622:Dapsone / Q000627:therapeutic use*; D003874:Dermatitis Herpetiformis / Q000175:diagnosis / Q000178:diet therapy / Q000276:immunology / Q000628:therapy*; D055050:Diet, Gluten-Free; D005260:Female; D006801:Humans; D007413:Intestinal Mucosa / Q000276:immunology / Q000473:pathology; D007421:Intestine, Small / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012867:Skin / Q000187:drug effects* / Q000276:immunology / Q000473:pathology; D013997:Time Factors; D016896:Treatment Outcome; D014945:Wound Healing; D055815:Young Adult
chemical_list: D003622:Dapsone