Dermatitis herpetiformis: jejunal findings and skin response to gluten free diet.

Abstract

SUMMARY Fifty seven children with dermatitis herpetiformis, 18 from Finland and 39 from Hungary, were studied. Diagnostic criteria included the finding of granular IgA deposits in the skin of all patients. The mean age at onset of the rash was 7-2 years and favoured sites were the elbows, knees, and buttocks. Symptoms suggesting small intestinal disease were rare but in 35 (61%) of the children subtotal villous atrophy and in 16 (28%) partial villous atrophy were found on jejunal biopsy.Eighteen children underwent a second biopsy after a mean of 21 months on a gluten free diet; villous height was found to be increased and the intraepithelial lymphocyte count decreased in all these patients. Gluten challenge caused a reversal in the two children who underwent a third biopsy. The effect of the gluten free diet on the rash was examined in Finnish children by observing the daily requirements of dapsone, a drug used to control the rash at the beginning of the diet. Eight (67%) of the 12 children were able to stop taking dapsone after a mean of 11 months on the diet and all three patients treated with diet alone became asymptomatic after three to 6 months on the diet.These results confirm that most children with dermatitis herpetiformis have jejunal villous atrophy, though they rarely have gastrointestinal symptoms. The central role of gluten in childhood dermatitis herpetiformis is evidenced by the fact that a gluten free diet helps the damaged jejunal mucosa to recover and controls the rash even in those children who do not have an abnormal jejunal biopsy.Dermatitis herpetiformis in adults is characterised by a pruritic, blistering rash and is associated with coeliac disease which is mostly subclinical in these patients.' Gluten plays an important role in adult dermatitis herpetiformis; both the enteropathy and rash respond to gluten withdrawal and relapse on gluten challenge.2-4 Dermatitis herpetiformis in

Gastrointestinal Symptoms Increase the Burden of Illness in Dermatitis Herpetiformis: A Prospective Study

“…In previous studies the prevalence of GI symptoms in DH cohorts has varied from 30% to none (3,(25)(26)(27). Since both patients' and dermatologists' primary focus is on the troublesome skin symptoms, there is a possibility that, especially milder, GI symptoms are ignored or not fully addressed.…”

Antipeptide Antibodies to Adenovirus E1b Protein Indicate Enhanced Risk of Celiac Disease and Dermatitis herpetiformis

“…Childhood celiac disease (CD) and dermatitis herpeti formis (DH) are activated by the gliadin fraction of gluten [1,2], and both the enteropathy and/or the skin lesions re cover following a gluten-free diet [3,4], In Finland both the incidence and clinical manifestations of childhood CD have altered during the past 15 years without major changes in population or gluten intake [5,6], Further more, on gluten challenge the time interval between the start of gluten intake and relapse of the enteropathy varies considerably from patient to patient [7,8], Thus, factors other than gluten intake may be involved in the etiopathogenesis of CD and DH.…”

Studies of Mucosal Immune Regulation in Celiac Disease and Type 1 Diabetes

“…DH is characterized by an itchy blistering skin rash associated with an increased density of IELs in response to gluten challenges [93]. About 75% of the patients with DH have small-bowel villous atrophy with crypt hyperplasia and the remainder have minor mucosal changes (reviewed in [94]).…”