Dermatitis herpetiformis: pathophysiology, clinical presentation, diagnosis and treatment

An Bras Dermatol. Nov-Dec 2014;89(6):865-75; quiz 876-7. doi: 10.1590/abd1806-4841.20142966.

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 gluten-free 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.

MeSH terms

  • Celiac Disease / etiology
  • Celiac Disease / pathology
  • Celiac Disease / therapy
  • Dapsone / therapeutic use
  • Dermatitis Herpetiformis / etiology
  • Dermatitis Herpetiformis / pathology*
  • Dermatitis Herpetiformis / therapy*
  • Diet, Gluten-Free
  • Female
  • Fluorescent Antibody Technique, Direct
  • Folic Acid Antagonists / therapeutic use
  • Humans
  • Male
  • Skin / pathology

Substances

  • Folic Acid Antagonists
  • Dapsone