Abstract: A 37-year-old male patient presented with an 8-year history of erythema, erosion and pain on bilateral inguinal regions, scrotum and perianal area. Histopathological examination of the skin lesions was consistent with the characteristics of familial chronic benign pemphigus (Hailey-Hailey disease). His father had a similar medical history. The patient's condition recurred frequently and responded poorly to various treatments. Based on previous literature reporting the use of dupilumab in the treatment of familial chronic benign pemphigus, dupilumab was administered to the patient. After 4 months of follow-up, the skin lesions were well-controlled without any adverse reactions observed. Different from previously reported cases which were mostly extensive, moderate-to-severe and refractory, the lesions in this case were mainly confined to the high-friction areas including bilateral inguinal regions, scrotum and perianal area, with a long and recurrent course of disease.

Key words: dupilumab, Hailey-Hailey disease