Abstract
Dermatitis herpetiformis (DH), a cutaneous manifestation of coeliac disease, is characterized by transglutaminase (TG) 3-targeted dermal immunoglobulin A (IgA) deposits. The treatment for DH is the same as for coeliac disease, namely a life-long gluten-free diet. DH patients typically have gluten-dependent circulating autoantibodies targeting TG3 and TG2, and plasma cells secreting such autoantibodies have been detected in the small intestinal mucosa. This study investigates the gluten-responsiveness of intestinal TG3 and TG2 antibody-secreting plasma cells in 16 treated DH patients undergoing a gluten challenge. The frequency of both plasma cell populations increased significantly during the challenge, and their frequency correlated with the corresponding serum autoantibody levels at post-challenge. TG3-specific plasma cells were absent in all 18 untreated coeliac disease patients and seven non-coeliac control subjects on gluten-containing diets. These findings indicate that, in DH, both intestinal TG3- and TG2-antibody secreting plasma cells are gluten-dependent, and that TG3-antibody secreting plasma cells are DH-specific.
Highlights
Dermatitis herpetiformis (DH), a cutaneous manifestation of coeliac disease, is characterised by an itching and blistering rash predominantly on the elbows, knees, and buttocks that arises in response to the ingestion of gluten-containing cereals, i.e., wheat, rye, and barley
TG3 and TG2 antibody-secreting plasma cells of all lamina propria plasma cells increased significantly. These plasma cell populations were not detected in all DH patients with a clear gluten-induced disease relapse, the findings strongly point to the gluten-dependency of these cells
The fairly short gluten exposure might have had an effect of the number of patients with the intestinal plasma cell subsets as well as the percentage of such cells, and a longer gluten intake might be required for all DH patients to react to a similar extent as in overt coeliac disease
Summary
Dermatitis herpetiformis (DH), a cutaneous manifestation of coeliac disease, is characterised by an itching and blistering rash predominantly on the elbows, knees, and buttocks that arises in response to the ingestion of gluten-containing cereals, i.e., wheat, rye, and barley. In the majority of DH patients, IgA-class anti-TG3 autoantibodies are found in the circulation [1,2]. The circulating TG3 autoantibodies are not entirely specific to DH, as approximately 30% of untreated coeliac patients have elevated levels of these autoantibodies in the absence of any skin symptoms [3,4]. The major autoantigen in coeliac disease is TG2, a member of the TG family along with TG3, and untreated patients characteristically have TG2-targeting autoantibodies (e.g., TG2 and endomysial antibodies, EmA) in the circulation and within various tissues, including the small intestine; as deposits at the subepithelial basement membrane and around the blood vessels [15,16]
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