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Three out of 4 patients with coexistent diabetes mellitus and dermatitis herpetiformis (DH) developed severe renal failure which resulted in death in 2 of them. Diabetes mellitus and DH may coexist more frequently than would occur by chance, and such patients may run an increased risk from severe renal complications of diabetes.
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Nine cases of lupus erythematosus (LE) associated with dermatitis herpetiformis (DH), seen at the Mayo Clinic, Rochester, Minn, were reviewed. The female to male ratio was seven to two. In seven patients DH developed first, followed by systemic LE in five and by discoid LE in two. In two patients systemic LE developed first, followed by DH. Since multiple autoimmune diseases have been reported in association with LE, and since similar HLA antigens have been found in patients with DH or LE, there is very likely an immunologic relationship between the two diseases.
Forty-five Hungarian and Finnish children from 1.5 to 15 years with dermatitis herpetiformis were studied for HLA antigens, jejunal morphology on gluten-containing diet and associated diseases in the patients and their relatives. A strong association with HLA-B8 was found in patients of both nationalities, the relative risks were 12.8 and 9.6, respectively. The Hungarian patients were also typed for HLA-DR locus, and an association with DR3 but not with DR7 was observed. Patients with subtotal villous atrophy had slightly more often HLA-B8 and DR3 than those with milder intestinal lesions. Atopic eczema occurred in 20% of the patients and family history of atopy seemed to have an inverse correlation with HLA-B8 and DR3.
Sera from 41 children with untreated celiac disease (CD), and 16 children with dermatitis herpetiformis (DH), and 57 matched controls were studied for serum antibodies to synthetic peptides derived from an early E1b protein of adenovirus type 12 and type 40 (Ad12, Ad40) and A-gliadin. In addition to peptides which share homology with A-gliadin, "nonhomologous" peptides derived from Ad12 and Ad40 E1b proteins were used as antigens in ELISA. Patients with CD and DH had significantly higher IgG antibody levels than those of controls to the nonhomologous Ad40 E1b peptide. Concomitant presence of antipeptide IgA antibodies to A-gliadin and Ad12 E1b or Ad40 E1b peptides (which share no homology with A-gliadin) increased the risk of CD/DH suggesting that both adenovirus infections and gluten exposure contribute to the development of CD/DH.
A 21-year-old man with coeliac disease and dermatitis herpetiformis presented successively with erosive ileitis (warranting surgical resection) and erosive jejunitis (proven by jejunoscopy). Discontinuous antibiotic therapy was associated with a gluten-free diet and evolution was favorable as judged with five years follow-up. Malabsorption with mucosal ulcerations can be due to: a) chronic ulcerative duodeno-jejuno-ileitis or Jeffries' disease; b) coeliac disease which may be classified as possible, probable, or certain according to the strictness of criteria. Our case is the fourth in which a villous response was proven after gluten-free diet. It is also particular in that the erosions were superficial; c) malignant lymphoma which can reasonably be excluded here. This observation confirms that a gluten-free diet may be effective after surgical resection of the ulcerated segment in complicated coeliac disease and shows that antibiotics may be an useful adjuvant to therapy.
Isopaque-Ficoll separated mononuclear cell suspensions from peripheral blood of patients with dermatitis herpetiformis (DH) and healthy controls were investigated by means of a rosette assay for Fc-receptor-bearing leukocytes (EA-RFC), peroxidase staining for monocytes (Pox) and a plaque formation assay (PFC) as well as a 51Cr release assay for cell-mediated cytotoxicity. The cell suspensions were investigated both before and after fractionation on nylon fibre columns. In the patients the mean percentage of PFC in unfractionated cell suspensions was significantly higher than in the controls. In fractionated cell suspensions both the mean percentage of EA-RFC and the mean cytotoxicity index in the 51Cr release assay were significantly lower than in the controls. There were no differences in the percentages of PFC- and Pox-positive cells in fractionated cell suspensions. The results suggest a numerical defect of circulating Fc-receptor-bearing lymphocytes as estimated both by the rosette assay and the 51Cr release assay. These data may reflect a pathogenetic role of these lymphocytes in DH.
The paper describes an adult patient with coeliac disease who developed classical dermatitis herpetiformis while receiving a gluten-free diet. The dermatitis is controlled by sulphapyridine but relapses when this is withdrawn, even though the patient continues with the diet, indicating that a gluten-free diet plays little or no part in controlling the skin condition.
Classical dermatitis herpetiformis (DH) is associated with similar HLA class I, II and III polymorphisms to coeliac disease (CD). The two diseases share distinctive pathological changes to the small intestinal mucosa which reverse on withdrawal of dietary gluten. In order to determine the locus primarily associated with DH, and to examine whether there is a common genetic link predisposing to the enteropathy seen in both DH and coeliac disease, HLA-DR, DQ and DP subregion associations were investigated by HLA genotyping in 23 DH patients and 64 healthy controls. We also studied polymorphisms of the TAP2 locus, which is located between the DP and DQ subregions. Genotyping was carried out by PCR of genomic DNA with allelic assignment by sequence-specific oligonucleotide (SSO) hybridization or amplification refractory mutation system (ARMS). The strongest associations in the patient group were with HLA DRB1*0301 (91% vs 22% of controls), HLA DQB1*02 (100% vs 32% of controls) and DPB1*0101 (39% and 14%). These associations are similar to those described for CD. 100% of DH patients were positive for the DQA1*0501/DQB1*02 dimer in cis or trans and, by analogy with CD, this is probably responsible for presenting gliadin peptide implicated in the disease process. Homozygosity for DQ2 was significantly increased in the CD patient group compared to the DH patient group (65% versus 39%), and so differences in dosage of HLA class II genotypes between DH and CD may be responsible for the milder gastrointestinal symptoms characteristic of DH.
Of two monozygotic twins, one had dermatitis herpetiformis, while the other had gluten-sensitive enteropathy. The literature on twins is reviewed.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Ultrastructural localization of in vivo-bound IgA in the skin of patients with dermatitis herpetiformis was determined by using a modified peroxidase-antiperoxidase multistep method. Three types of reaction product deposition are seen. The most common type of reaction product deposition, that which is identified by direct immunofluorescence as the speckled type of IgA deposit, shows up as clumps and yarnlike fibrils. The second type of IgA deposition, which is a linear band by direct immunofluorescence, appears to be associated with anchoring fibrils. The third of IgA deposition, which is also linear by immunofluorescence, is confined to the lamina lucida.