Dermatitis herpetiformis (DH) is a chronic, pruritic autoimmune blistering disorder associated with gluten-sensitive enteropathy. DH can rarely present in childhood, with slight female predilection, in contrast to adult DH, which shows male predilection. The pathogenesis involves the deposition of IgA immune complexes in the papillary dermis. The associated autoantigen is an epidermal transglutaminase (transglutaminase 3, or TG3). There is a genetic predisposition to the disease, as it is associated with certain human leukocyte antigen (HLA) haplotypes, HLA class II DQ2.

The cutaneous lesions seen in childhood are similar to those in adults, with symmetric papulovesicular eruption on the extensor surfaces and buttocks that is extremely pruritic and may wax and wane in severity. Facial involvement has been reported. Pruritus and nonpruritic, noninflammatory papules and pruritic purpuric macules / petechiae on the palms can be a rare presentation with or without the typical papulovesicular eruption.

About 80%-95% of children with DH have gluten-sensitive enteropathy on small intestine biopsy. Most are asymptomatic and undiagnosed at presentation of DH; only 10% of children carry a previous diagnosis of celiac disease.

There is an increased incidence of autoimmune diseases in patients with DH. The most common associated autoimmune conditions include thyroid dysfunction, particularly Hashimoto thyroiditis, type 1 diabetes mellitus, and pernicious anemia. Patients with DH are at an increased risk of developing enteropathy-associated T-cell lymphoma. However, a gluten-free diet may be protective against cancer development.