title: Extramammary Paget disease in peristomal skin: report of a unique case.
abstract: OBJECTIVE
  Extramammary Paget disease is a rare neoplasm most often found in the perianal area which can be primary in origin or secondary from underlying colorectal adenocarcinoma with epidermotropic spreading. However, the occurrence of extramammary Paget disease has not been described in peristomal skin.
  
  
  METHODS
  We describe a single case of Paget disease in the peristomal skin of a 61-year-old woman with ulcerative colitis who underwent a proctocolectomy 33 years prior to now with chronic intestinal obstruction and an area of "ectopic mucosa" adjacent to her ileostomy. The patient was taken to surgery for exploratory laparotomy and revision of her stoma.
  
  
  RESULTS
  Macroscopic and microscopic examinations of the peristomal lesion demonstrated Paget disease with intraepidermal spreading of dysplastic intestinal/glandular cells but no invasive adenocarcinoma in the peristomal skin after extensive sampling.
  
  
  CONCLUSIONS
  To our knowledge, this is the first reported case of extramammary Paget disease occurring in peristomal skin. The current case emphasizes the importance of recognizing such disease and the potential for associated neoplasia in patients with intestinal stomas.
authors.affiliation: Department of Anatomic Pathology, Cleveland Clinic, 9500 Euclid Avenue / L25, Cleveland, OH 44195, USA. liux3@ccf.org
authors.forename: Xiuli
authors.initials: X
authors.lastname: Liu
authors.forename: Genevieve B
authors.initials: GB
authors.lastname: Melton
authors.forename: Hao
authors.initials: H
authors.lastname: Xie
authors.forename: David W
authors.initials: DW
authors.lastname: Dietz
mesh_terms: D003093:Colitis, Ulcerative / Q000601:surgery*; D005260:Female; D006801:Humans; D007081:Ileostomy; D008875:Middle Aged; D010145:Paget Disease, Extramammary / Q000175:diagnosis* / Q000209:etiology; D011183:Postoperative Complications / Q000175:diagnosis*; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000209:etiology; D054047:Surgical Stomas / Q000473:pathology*