Dermatitis Herpetiformis

Key Points
Intensely itchy rash consisting of small blisters and bumps
Usually chronic, with periodic exacerbations

Dermatitis herpetiformis, also known as Duhring’s Disease, is an intensely itchy rash made up of bumps and blisters. These are tiny fluid-filled blisters, however the surface is often rubbed or scratched off leaving a red sore or bump. The bumps or blisters usually appear on the elbows, knees, back, and buttocks. In most cases, the rash is the same size and shape on both sides. Symptoms of Dermatitis Herpetiformis are intermittent, though the condition is chronic, meaning that it can last a long period of time. The condition usually starts between the ages of 15 and 40, though it can affect children and the elderly.

The skin lesions are caused by the deposition of IgA(an Immunoglobulin protein of the immune system) in the papillary dermis, which triggers an immunologic cascade, resulting in inflammation. The IgA which is deposited in the skin is the result of an immunologic response to chronic stimulation of the gut mucosa by dietary gluten.

Differential Diagnosis (Other conditions with similar appearance)

Bullous pemphigoid
Erythema multiforme
Neurotic excoriations
Scabies
Transient Acantholytic Dermatosis

Diagnosis
Diagnosis is by clinical exam with blood tests and skin biopsies, which detect the level and pattern of IgA (Immunoglobulin A) antibodies.

Treatment
Dapsone in conjunction with a gluten free diet
The condition cannot be cured or prevented unfortunately

Dermatitis herpetiformis is a lifelong condition, though it may go in remission for some victims. Although there is no cure, and though it can’t be prevented, the disease can be controlled with proper treatment. Treatment includes elimination of gluten from the diet usually with Dapsone. Dapsone has historically been an empiric test for Dermatititis Herpetiformis, as rapid relief of itching and lesions is usually noted with administration. In rare cases, patients with long standing, poorly controlled disease may be at increased risk for intestinal lymphoma.