A case of quadruple extramammary Paget's disease

Abstract

Herein, we report a rare case of quadruple extramammary Paget's disease (EMPD) affecting four parts of the body (the genitals, both axillae, and the umbilical region). Four lesions were present in the genital area (on the suprapubic area, scrotum, penile shaft, and glans penis). An 88-year-old Japanese man presented with a 10-year history of a skin rash on the right inguinal area. A physical examination revealed a large tumor with a papillomatous appearance (size : 17×14 cm) in the suprapubic region, a slightly elevated plaque (size : 6.5×4.5 cm) on the scrotum, an erythematous tumor (size : 5×5 cm) on the penile shaft, and depigmentation on the glans penis. Furthermore, erythematous patches were observed on both axillae and the umbilicus. Biopsy examinations revealed Paget's cells in all lesions, except in the glans penis lesion. Histological examinations of the excised lesions did not reveal any invasive findings. After 1 year and 4 months, an erythematous tumor developed on the glans penis, where depigmentation had been previously observed, and he was diagnosed with EMPD. The patient subsequently developed rectal carcinoma 4 years later, and died from respiratory failure after the operation.[Skin Cancer (Japan) 2017 ; 32 : 272-278]