Dermatitis Herpetiformis

Abstract

The state of our understanding of the pathogenesis of DH relies on the integration of several key characteristics: (1) a high frequency of the HLA antigens HLA-B8, HLA-DR3, and HLA-DQw2, (2) an associated GSE, (3) the resolution of both the skin lesions and gut abnormalities in response to a gluten-free diet, and (4) the presence of granular deposits of IgA in normal and perilesional skin. The role of the HLA class II antigens expressed in patients with DH most likely relates to the afferent or initiating arm of the immune system. The association of the HLA-A1, -B8, -DR3, -DQw2 haplotype with Sjogren's syndrome, chronic hepatitis, Graves' disease, and other presumably immunologically mediated diseases, as well as the evidence that some normal HLA-B8, -DR3 individuals have an abnormal in vitro lymphocyte response to wheat protein and mitogens and have abnormal Fc-IgG receptor-mediated functions, suggests that this HLA haplotype or genes linked closely to it may confer a generalized state of immune susceptibility on its carrier, the exact phenotypic expression of which depends on other genetic or environmental determinants. It also is clear, from the association of DH with GSE and the ability to control the cutaneous manifestations of DH with a gluten-free diet, that the gut disease is a critical factor in the pathogenesis of DH. Several pathogenetic theories about the origin of the cutaneous IgA deposits in DH have been proposed, one of which states that the IgA is produced in the gut mucosa as a response to a dietary antigen or gut epithelial antigen and then cross-reacts with the skin of patients with DH. A second hypothesis is that the IgA produced in the gut binds to an antigen and is deposited in skin as an antigen-antibody complex. Finally, it could be that the gut mucosal abnormality simply allows an unknown antigen access to the central immune system where an IgA antibody is produced that binds to skin. The failure to detect circulating IgA anti-basement membrane zone antibodies in patients with DH suggests that either the structures to which the IgA binds are not present in normal skin without DH, that IgA cannot bind to these structures in vitro, or that the circulating IgA is too scant for detection with conventional methods. Finally, it must be considered that the IgA deposited in DH skin may bind as a result of non-antigen-antibody interactions that cannot be duplicated in vitro.(ABSTRACT TRUNCATED AT 400 WORDS)

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

“…Similar negative findings have been recorded in the lesion-free, blister-resistant skin of DH patients (26). However, others have observed decreased amounts of IgA deposits in uninvolved skin of DH patients (27,28). Biopsies were also performed on blistered (infected) tails, and no IgA deposits were found.…”

Celiac Sprue

“…The diagnosis requires the demonstration by immunofluorescence studies of granular deposits of IgA in an area of normal-appearing skin. 26 A smallbowel biopsy in patients with dermatitis herpetiformis demonstrates a mild and patchy gluten-sensitive enteropathy. The skin lesions respond to the withdrawal of gluten from the diet or to treatment with dapsone.…”

Flare of dermatitis herpetiformis associated with gluten in multivitamins

“…There are only 5 reported cases of SS after vaccination. [1][2][3][4] Our case would be the first following influenza vaccination.…”