Dermatitis herpetiformis successfully treated with dupilumab

Dermatitis herpetiformis (DH) is an autoimmune blistering disease characterized by pruritic papulovesicular lesions found mostly on extensor surfaces. The cutaneous manifestation is caused by an immunologic response to dietary gluten, leading to the formation of immunoglobulin A antibodies against tissue transglutaminase (TG) and epidermal TG 3 that ultimately deposit in the papillary dermis.1 Therapeutic approaches combine a gluten-free diet along with dapsone to control the debilitating nature of the cutaneous and intestinal manifestations of DH. Limited data exists regarding second-line treatments of DH, which makes it challenging to treat those patients with contraindications to dapsone.2 Dapsone is metabolized by the liver via acetylation and hydroxylation, producing oxidative metabolites that can exacerbate hemolysis and methemoglobinemia in high-risk patients; thus, it is preferably avoided in patients with glycogen storage disease (GSD), due to their impaired hepatic metabolism and increased risk of oxidative stress.3 This is a novel case report demonstrating the successful treatment of DH with dupilumab in a 21-year-old female. Due to an underlying GSD, dapsone was contraindicated, and she was effectively managed with dupilumab 300 mg every 2 weeks.