Chapter 75
Childhood Dermatitis Herpetiformis
Carmen Liy Wong,
Irene Lara-Corrales,
Carmen Liy Wong
Section of Dermatology, Division of Paediatric Medicine, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada
Search for more papers by this authorIrene Lara-Corrales
Section of Dermatology, Division of Paediatric Medicine, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada
Search for more papers by this authorCarmen Liy Wong,
Irene Lara-Corrales,
Carmen Liy Wong
Section of Dermatology, Division of Paediatric Medicine, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada
Search for more papers by this authorIrene Lara-Corrales
Section of Dermatology, Division of Paediatric Medicine, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada
Search for more papers by this authorBook Editor(s):Peter Hoeger,
Veronica Kinsler,
Albert Yan,
John Harper,
Arnold Oranje,
Christine Bodemer,
Margarita Larralde,
David Luk, Vibhu Mendiratta,
Diana Purvis,
Peter Hoeger
Search for more papers by this authorVeronica Kinsler
Search for more papers by this authorAlbert Yan
Search for more papers by this authorJohn Harper
Search for more papers by this authorArnold Oranje
Search for more papers by this authorChristine Bodemer
Search for more papers by this authorMargarita Larralde
Search for more papers by this authorVibhu Mendiratta
Search for more papers by this authorDiana Purvis
Search for more papers by this authorFirst 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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