期刊
JOURNAL OF CLINICAL INVESTIGATION
卷 114, 期 8, 页码 1090-1097出版社
AMER SOC CLINICAL INVESTIGATION INC
DOI: 10.1172/JCI200421055
关键词
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资金
- NIAID NIH HHS [R01 AI014764, R37 AI014764, AI14764] Funding Source: Medline
- NIDDK NIH HHS [DK57892, R01 DK057892] Funding Source: Medline
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.
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