A new model for dermatitis herpetiformis that uses HLA-DQ8 transgenic NOD mice

Eric Marietta, Kay Black, Michael Camilleri, Patricia Krause, Roy S. Rogers, Chella David, Mark R. Pittelkow, Joseph A. Murray

Research output: Contribution to journalArticlepeer-review

115 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)1090-1097
Number of pages8
JournalJournal of Clinical Investigation
Issue number8
StatePublished - Oct 2004

ASJC Scopus subject areas

  • General Medicine


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