Dermatitis herpetiformis: diagnosis, diet and demography.

Abstract

SUMMARY We describe a long term study of 76 patients with dermatitis herpetiformis. Unlike patients with coeliac disease, where the peak incidence was during the first and fourth decades, no dermatitis herpetiformis patients presented in the first decade; also, there was a male preponderance in dermatitis herpetiformis which contrasts with the excess of females in coeliac disease. The apparent prevalence of dermatitis herpetiformis was 11 per 100 000 in our population; approximately one fifth of that of coeliac disease. Jejunal villous atrophy was present in 78% of our dermatitis herpetiformis patients, and a single jejunal biopsy was as effective at detecting this as the multiple biopsy technique. A majority of patients were able to stop, or radically reduce their dapsone or sulphapyridine treatment after the institution of a gluten free diet. Spontaneous remission of the skin lesion occurred in only two patients not receiving a gluten free diet. Gastric parietal or thyroid antibodies were detected in 38% of patients, and three cases of thyroid disease and two cases of pernicious anaemia were detected. Lymphoma developed in two patients, one being intestinal in origin. We conclude that a gluten free diet is of therapeutic benefit in dermatitis herpetiformis and that spontaneous remission is uncommon in those not on a diet. Despite patchiness of the enteropathy, a single jejunal biopsy is quite adequate to diagnose the presence of upper intestinal villous atrophy.Since the first report of the association of dermatitis herpetiformis with coeliac disease in 1966,1 evidence of close links between the two disorders has accumulated. Well documented points of similarity include the response of the enteropathy to a gluten free diet,23 the high prevalence of HLA B8 and DR3 phenotypes, the occurrence of hyposplenism78 and a predisposition to the development of lymphoma.9 10 While these associations are not in dispute, controversy has arisen in relation to the major differences between the two disorders. By definition, the appearance of the skin lesions characterises dermatitis herpetiformis, but, while some authors believe that exclusion of gluten from the diet leads to remission of the skin disease 11-14 others disagree.5 16 Similarly, there is disagreement about the frequency with which intestinal villous

Gluten related disorders: even a dermatological issue

“…In particular, the estimated prevalence rate was 75.3 per 100,000, while annual incidence rates were respectively 5.2 per 100,000 in 1980-1989, 2.9 per 100,000 in 1990-1999 and 2.7 per 100,000 in 2000-2009, with a decrease in incidence rate between the first and second 10-year period that was statistically significant. In the study of Salmi et al (14) emerged a ratio between DH and CD of 1:8, that resulted lower than 1:5 showed in previous studies (15). Theoretically, the risk of a celiac patient to develop DH remains high, but, being the diagnosis of the enteropathy and therefore the adoption of GFD ever earlier, the risk of DH is drastically reduced (14) as postulated also by Fry (16).…”

Dermatitis herpetiformis misdiagnosed and treated as tinea cutis glabrae

“…Obserwacje kliniczne potwierdzają obecność zmian chorobowych typowych dla choroby Duhringa u 12% pacjentów z rozpoznaną chorobą trzewną [4]. U chorych z opryszczkowatym zapaleniem skóry rzadko jednak występują objawy żołądkowo-jelitowe, a mimo to u 75% z nich stwierdza się zanik kosmków w jelicie cienkim [5,6]. Badania serologiczne mają charakter pomocniczy w ustalaniu rozpoznania opryszczkowatego zapalenia skóry.…”

The Gut in Skin Disease: Gastro-Intestinal Manifestations of Selected Skin Disorders

“…Both DH and coeliac disease are glutenrelated disorders, and about 75% of patients with DH and villous atrophy are improved by a gluten-free diet [11], even though the cutaneous lesions recover only slowly. It is not known whether gluten avoidance alters the recently recognised risk of intestinal lym phoma [16], although it has not yet been shown to prevent it in coeliac disease.…”

“…when demonstrated by immunofluores cence, are a distinctive finding. About three quarters of patients with DH have partial or subtotal villous atrophy on jejunal biopsy [11], although some investigators have found an even higher prevalence of abnormalities if raised intra-epithelial lymphocyte counts are included [12]. The intestinal lesion of DH is patchy, changes varying from near normality to complete atrophy [13], but despite this a single biopsy is diagnostically adequate [11].…”