Short-term disease control with alternate-day maintenance upadacitinib in refractory Hailey-Hailey disease: a case...

Hailey-Hailey disease (HHD), also known as benign familial pemphigus, is a rare autosomal dominant genodermatosis characterized by recurrent erosive lesions in intertriginous areas. Previous case reports have described clinical improvement with upadacitinib, but post-response maintenance strategies remain poorly defined. We report a 62-year-old woman with a 7-year history of refractory HHD who showed marked improvement after 2 months of upadacitinib 15 mg once daily. After 6 months of daily treatment, the lesions had completely cleared and disease control was stable. The regimen was then changed to 15 mg every other day after discussion with the patient. The change was based on sustained clinical control, concern about the financial burden of continued daily treatment, and a preference to limit prolonged systemic exposure; it was not prompted by an adverse event. The patient adhered to the alternate-day schedule, did not resume daily treatment, and required no rescue or additional therapy. During the subsequent 3 months, no new or recurrent lesions were observed and no apparent adverse events occurred. This case therefore documents short-term disease control after transition to an alternate-day maintenance schedule. Given the relapsing and fluctuating course of HHD and the limited follow-up after dose reduction, the observation should not be interpreted as evidence of durable remission or long-term efficacy.