Dermatitis herpetiformis: pathophysiology, clinical presentation, diagnosis and treatment

Abstract

Researches on DH have shown that it is not just a bullous skin disease, but a cutaneous-intestinal disorder caused by hypersensitivity to gluten. Exposure to gluten is the starting point of an inflammatory cascade capable of forming autoantibodies that are brought to the skin, where they are deposited, culminating in the formation of skin lesions. These lesions are vesico-bullous, pruritic, and localized especially on elbows, knees and buttocks, although atypical presentations can occur. Immunofluorescence of perilesional area is considered the gold standard for diagnosis, but serological tests help in cases where it is negative. Patients who follow glutenfree diets have better control of symptoms on the skin and intestine, as well as lower risks of progression to lymphoma. Dapsone remains the main drug for treatment, but it requires monitoring of possible side effects, some potentially lethal.

Dermatitis herpetiformis: celiac disease of the skin. Report of two cases

“…In pathogenesis, HLA DQ2 and HLA DQ8 haplotypes are known to be involved, both in dermatitis herpetiformis and in celiac disease [7,8]. 90% of patients are DQ2 positive, and the remaining DQ8 [5,6].…”

“…In addition, excoriations, hyperpigmented scars may be present, because these lesions have periods of remission [1][2][3][4][5][6][7][8][9][10].…”

“…In children, it is common to find petechial or echimotic lesions in palmoplantar regions, especially at fingertips [6,7]. Mucosal involvement is rare, although IgA is frequently deposited in these regions [7].…”

“…Mucosal involvement is rare, although IgA is frequently deposited in these regions [7]. Other less common forms are also seen as palmoplantar hyperkeratosis, purupuric lesions with the appearance of leukocytoclastic vasculitis and lesions that mimic prurigo pigmentosum [7,11].…”

“…Tissue anti-transglutaminase antibodies are elevated in patients with intestinal activity and decreases with the adoption of a gluten free diet [7].…”