Abstract: We report a patient with refractory Hailey-Hailey disease (HHD) who had a disease course of more than 40 years and failed conventional treatments including minocycline and acitretin. The patient received combination therapy of upadacitinib and acitretin, with remarkable relief of skin lesions at week 4 and nearly complete resolution of lesions at month 2. The patient was followed-up for 6 months with no recurrence. Literature review revealed that there are only 14 case reports regarding the application of JAK inhibitors in HHD at present. All types of JAK inhibitors can rapidly ameliorate skin lesions with mild short-term adverse reactions, while large-sample and long-term clinical studies are still lacking.

Key words: familial benign chronic pemphigus, Hailey-Hailey disease, upadacitinib, acitretin