Treatment of Recalcitrant Hailey-Hailey Disease with Dupilumab: A Case Report.

Abstract

Hailey-Hailey disease is a rare autosomal dominant genodermatosis clinically characterized by recurrent flaccid bullae, erosive plaques, and linear epidermal tears primarily affecting intertriginous areas. This case report describes a 34-year-old White female with a 15-year history of severe, treatment-refractory Hailey-Hailey disease affecting multiple intertriginous areas. After failing numerous topical and systemic therapies, the patient initiated dupilumab therapy with a 600mg loading dose followed by 300mg administered every two weeks as maintenance. Within four months, the patient demonstrated approximately 80 percent clinical improvement with significant reduction in pain and pruritus. The authors speculate dupilumab's efficacy may extend beyond calcium homeostasis regulation, potentially addressing an unrecognized autoimmune component of Hailey-Hailey disease wherein desmosomal disruption may expose normally protected epitopes to immune surveillance. The rapid clinical response to dupilumab in this previously treatment-refractory case warrants further investigation into the immunopathogenesis of Hailey-Hailey disease and supports dupilumab as a promising therapeutic option for this debilitating condition.