title: Perianal extramammary Paget's disease associated with primary linitis plastica of the rectum: report of a case.
abstract: We report herein a case of perianal extramammary Paget's disease associated with primary linitis plastica of the rectum. An 82-year-old woman was admitted to our hospital for investigation and treatment of a perianal eczematous lesion. A skin biopsy of the lesion revealed perianal extramammary Paget's disease and a barium enema demonstrated diffuse narrowing with an irregular contour at the ampulla recti. Under a suspected diagnosis of linitis plastica of the rectum, an abdominoperineal resection was performed to resect both the rectal and perianal lesions with regional lymphadenectomy. Grossly, marked narrowing and wall thickness were observed at the lower rectum, and a histological diagnosis of signet ring cell carcinoma was confirmed. The perianal eczematous lesion revealed many atypical cells with clear cytoplasm, being Paget cells, throughout the entire epidermis. Sparse distributions of signet ring cells were also observed in the subcutaneous tissue beneath the perianal eczematous lesion. The pathogenesis of perianal extramammary Paget's disease in this patient was therefore considered to be an intraepidermal extension of primary linitis plastica of the rectum.
authors.affiliation: Second Department of Surgery, Kagoshima University, Faculty of Medicine, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Ikoma
authors.forename: H
authors.initials: H
authors.lastname: Kubo
authors.forename: T
authors.initials: T
authors.lastname: Ohno
authors.forename: K
authors.initials: K
authors.lastname: Tanaka
authors.forename: A
authors.initials: A
authors.lastname: Yoshida
authors.forename: A
authors.initials: A
authors.lastname: Taira
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001005:Anus Neoplasms / Q000150:complications* / Q000473:pathology; D018279:Carcinoma, Signet Ring Cell / Q000150:complications* / Q000473:pathology; D005260:Female; D006801:Humans; D008039:Linitis Plastica / Q000150:complications* / Q000473:pathology; D010145:Paget Disease, Extramammary / Q000150:complications* / Q000473:pathology; D012004:Rectal Neoplasms / Q000150:complications* / Q000473:pathology