Dermatitis herpetiformis (DH) is an uncommon autoimmune skin disorder that causes an itchy skin rash and is commonly associated with Coeliac Disease. It also gets called Duhring disease (after Louis Duhring in 1884) or Duhring–Brocq disease. More than 90% of those with dermatitis herpetiformis have Coeliac disease and 10-20% of those with Coeliac disease develop dermatitis herpetiformis. It most commonly occurs bewteen the age of 15 to 40 years.
Aetiology:
Dermatitis herpetiformis and coeliac disease are due a reaction to the gliadin fraction of gluten that is found in wheat, rye and barley that triggers production of IgA antibodies. The autoimmune process then targets the skin (dermatitis herpetiformis) or the intestinal wall (coeliac disease). A genetic predisposition underlies both conditions.
Clinical Features:
Symmetrical distribution of very itchy papules and vesicles mostly on the scalp, shoulders, buttocks, and extensor surfaces of the elbows and knees. The eruption is a waxing and waning one.
The lesions resemble those caused by the herpes virus (hence the name, herpetiformis), but they are not caused by the virus.
The skin lesions resolve to leave postinflammatory hypopigmentation and hyperpigmentation.
There may also be the gastrointestinal symptoms suggestive of Coeliac disease.
Diagnosis is typically done via a skin biopsy and blood serology for the Coeliac markers.
Differential diagnosis: eczema; bullous pemphigoid; erythema multiforme; scabies
Management:
Gluten-free diet
Oral Dapsone for the itch; and maybe topical corticosteroids.
Prognosis is good if adhere to a gluten-free diet.
Page last updated:
Comments are closed.