Abstract
Hailey-Hailey disease (HHD) is an autosomal dominant blistering dermatosis with incomplete penetrance caused by an ATP2C1 gene mutation. Currently, there is no cure for HHD; however therapeutic options aim to minimize the exacerbating factors and manage patients’ symptoms. A 58-year-old male presented with a 10-year history of biopsy-proven HHD. He was seen consistently over the course of nine years with multiple flares a year consisting of pruritic and painful intertriginous plaques with involvement of the chest, upper arms, and back. His disease was recalcitrant to topicals, oral and topical antibiotics, phototherapy, and systemic corticosteroids. He was started on dupilumab and noticed significant improvement. We present a case of HHD recalcitrant to various modalities of treatment. Our description of rapid improvement with dupilumab suggests a role for Th2 signaling in the pathophysiology of HHD. We propose that dupilumab works for HHD due to the significant skin barrier dysfunction, similar to atopic dermatitis, and consider whether inflammation plays an earlier role in the disease. Although used for an off-label purpose, in this case, further studies should assess the clinical response and safety of patients with recalcitrant HHD treated with dupilumab.
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