Dermatitis herpetiformis

Published on 11/06/2015 by admin

Filed under Pediatrics

Last modified 22/04/2025

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Grouped blisters and erosions around the elbow in a child with DH.

Grouped blisters on the dorsum of the hand in a child with DH.

CLINICAL FEATURES

In dermatitis herpetiformis (DH) IgA antibodies are directed at epidermal transglutaminase. All patients with DH have gluten-sensitive enteropathy, although it may be mild or asymptomatic. DH is characterized by severely pruritic grouped papules, vesicles or urticarial lesions. Patients usually become symptomatic in the adolescent years.

Classic sites of predilection for DH are the elbows, knees, buttocks and upper trunk, but lesions may occur anywhere including the scalp.

Urticarial lesions are common and DH may initially be misdiagnosed as chronic urticaria. Pruritus is often so severe that intact vesicles are not seen and the patient presents with only excoriations.

Biopsy of perilesional skin with immunofluorescence evaluation reveals IgA in the dermal papillae. DH is most prevalent in Northern European populations and may be associated with the development of lymphoma in adult life.

TREATMENT

Patients respond within weeks to a few months to a gluten free diet, although it is difficult for a child to follow rigorously.

Dapsone 0.5 to 2.0 mg/kg/day is an effective treatment, but it is associated with the risk of serious side effects including methemoglobinemia, hemolysis and hypersensitivity syndrome. Before starting dapsone, serum glucose 6 dehydrogenase, compete blood count and liver function tests should be evaluated. CBC and LFTs should be followed throughout therapy, which is life long.

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