A 92-year-old man with dementia presented with perianal discomfort and fecal incontinence of 1 week duration. He denied pain, pruritus, bleeding, or sensation of a mass. Physical examination was notable for a raised verrucous mass (0.75 cm) extending from the dentate line distally for 7 cm, circumferentially covering 75% of the anal margin. Punch biopsy specimens were obtained for diagnosis. Histology revealed intraepithelial cells with prominent nucleoli and abundant clear cytoplasm. Surgical options were explained to the patient, but given his decline in function and overall poor life expectancy, radiation therapy was offered. He successfully completed a regimen of 44 Gy in 11 fractions. On follow-up examination, no appreciable perianal mass was observed; the patient denied anal discomfort, pain, bleeding, or symptoms of obstruction.
Perianal Paget disease is uncommon, with fewer than 300 cases reported in the literature.
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Classically described as involving the breast, Paget disease has been shown to affect areas containing apocrine glands, including the perianal region, vulva, penis, scrotum, thighs, buttocks, and axilla.2
Perianal Paget disease typically presents in the sixth decade of life with pruritus ani, bleeding, excoriation, pain with defecation, anal pain, or mass sensation.2
Lesions are characterized as slightly raised, erythematous, and well-demarcated. Treatment is predominantly surgical, consisting of local excision, wide local excision with skin grafting or flap reconstruction, or in severe cases abdominoperineal resection.4
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Radiation therapy can be offered in select patients with good outcome.REFERENCES
- Sur un cas de maladie de Paget de la region perinea-anale et scrotale.Ann Dermatol Syph. 1893; 4: 25-33
- Radiotherapy for perianal Paget's disease: a case report.Tumori. 2008; 94: 750-753
- Case report: perianal Paget's disease.Br J Radiol. 1999; 72: 610-612
- Surgical treatment of extramammary Paget's disease: a report of six cases and a reexamination of Mohs micrographic surgery compared with conventional excision.Cancer. 1991; 67: 933-938
- Perianal Paget's disease, classification and review of management. Report of 2 cases.Dis Colon Rectum. 1990; 33: 502-507
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© 2011 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.
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