Open AccessA new model for dermatitis herpetiformis that uses HLA-DQ8 transgenic NOD mice
Author(s) -
Eric Marietta,
Kay E. Black,
Michael Camilleri,
Patricia K. Krause,
Roy S. Rogers,
Chella S. David,
Mark R. Pittelkow,
Joseph A. Murray
Publication year - 2004
Publication title -
the journal of clinical investigation/the journal of clinical investigation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 6.278
H-Index - 488
eISSN - 1558-8238
pISSN - 0021-9738
DOI - 10.1172/jci200421055
Subject(s) - dermatitis herpetiformis , nod , dapsone , enteropathy , immunology , medicine , gluten , rash , pathogenesis , nod mice , autoimmunity , dermatology , pathology , disease , immune system , diabetes mellitus , endocrinology
Dermatitis herpetiformis (DH) is an autoimmune blistering skin disorder that is associated with gluten sensitivity. It presents as a papulovesicular rash and is often associated with enteropathy. The rash resolves when the patient is placed on a gluten-free diet and/or dapsone. DH, as well as celiac disease, is tightly associated with DQ2 and DQ8. A novel mouse model for DH is described that utilizes the NOD background and the HLA-DQ8 transgene. The addition of DQ8 contributes sensitivity to gliadin, and the addition of the NOD background contributes to autoimmunity and pathogenesis. Fifteen NOD DQ8+ mice of 90 that were sensitized to gluten developed blistering pathology similar to that seen in DH. Neutrophil infiltration of the dermis, deposition of IgA at the dermal-epidermal junction, and a complete reversal of the blistering phenomenon with the administration of a gluten-free diet with or without dapsone were observed. None of the 3 blistering mice examined had small-bowel pathology. This animal model of DH will be useful to determine the specificity of the IgA deposits, as well as the pathogenic mechanisms that occur in the skin as a result of gluten ingestion