PERIANAL EXTRAMAMMARY PAGETS-DISEASE ASSOCIATED WITH PRIMARY LINITIS PLASTICA OF THE RECTUM - REPORT OF A CASE

We report herein a case of perianal extramammary Paget's disease assoc iated with primary linitis plastica of the rectum, An 82-year-old woma n was admitted to our hospital for investigation and treatment of a pe rianal eczematous lesion, A skin biopsy of the lesion revealed periana l extramammary Paget's disease and a barium enema demonstrated diffuse narrowing with an irregular contour at the ampulla recti, Under a sus pected diagnosis of linitis plastica of the rectum, an abdominoperinea l resection was performed to resect both the rectal and perianal lesio ns with regional lymphadenectomy. Grossly, marked narrowing and wall t hickness were observed at the lower rectum, and a histological diagnos is of signet ring cell carcinoma was confirmed, The perianal eczematou s lesion revealed many atypical cells with dear cytoplasm, being Paget cells, throughout the entire epidermis, Sparse distributions of signe t 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 int raepidermal extension of primary linitis plastica of the rectum.