Perianal Paget's disease : case report and review of the literature

Cutaneous Paget's disease (PD) is a rare entity, predominantly involving the breasts. Anal involvement is rather exceptional, and can be associated with underlying malignancies, among which prostate and rectal adenocarcinoma. We report the case of a 71- year-old man suffering from a long history of anal itching, associ- ated with an erythematosquamous lesion of the right buttock extending up to the anus. The diagnosis of perianal PD (PAPD) was confirmed by histopathological demonstration of Paget's cells from a biopsy performed after ineffective topical treatment. Radiological and further clinical inspections allowed us to exclude any synchronous malignancy. A first-step surgery consisted in coe- lioscopic diverting sigmoid colostomy, along with multiple peri- anal, anal and rectal biopsies revealing an anal canal involvement. Coelioscopic abdominoperineal surgery and a wide cutaneous excision were then performed. Histopathological analysis revealed positive posterior margin, requiring further excision. No adjuvant therapy was prescribed, and to this day, after a one-year and a half follow-up, the patient remains disease-free. Our case report and review of PAPD stress that appropriate management is required to improve the poor prognosis of this rare affection. (Acta gastroenterol. belg., 2004, 67, 228-231).