Chapter 75

Childhood Dermatitis Herpetiformis

Carmen Liy Wong

Carmen Liy Wong

Section of Dermatology, Division of Paediatric Medicine, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada

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Irene Lara-Corrales

Irene Lara-Corrales

Section of Dermatology, Division of Paediatric Medicine, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada

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First published: 20 November 2019

Summary

Dermatitis herpetiformis (DH) is an inflammatory cutaneous disease with a chronic relapsing course, pruritic polymorphic lesions and typical histopathological and immunopathological findings. It is now considered the specific cutaneous manifestation of coeliac disease (CD). DH usually presents with symmetrical, grouped, erythematous papules, urticarial plaques, vesicles and secondary excoriations involving the extensor surfaces of the knees, elbows, shoulders, buttocks, sacral region, neck, face and scalp. Typical histopathological findings consist of fragile, subepidermal vesicles with accumulation of neutrophils at the papillary tips. Direct immunofluorescence is the gold standard for diagnosis and shows granular IgA deposition at the dermal papillae. Positive IgA antitissue transglutaminase (tTG), IgA antiendomysium antibodies (EMAs) and IgA antiepidermal transglutaminase (eTG) are considered specific and sensitive serological markers for DH. Both DH and CD occur in gluten-sensitive individuals, share the same HLA haplotypes (DQ2 and DQ8) and improve following administration of a gluten-free diet (GFD). Although life-long GFD is the treatment of choice for DH, it takes a long time for the cutaneous manifestations to resolve; thus, dapsone is highly recommended during the first 12–24 months of treatment. Patients with DH should be carefully monitored due to the association with intestinal malabsorption, autoimmune diseases and/or lymphoma.

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