Ann Surg Treat Res.  2017 Dec;93(6):336-341. 10.4174/astr.2017.93.6.336.

Perianal Paget disease: a report of 2 cases

• Park YY
• Kim M
• Cheong C
• Kim SK
• Song SY
• Chung KY
• Kim NK

• KMID: 2396876
• DOI: http://doi.org/10.4174/astr.2017.93.6.336

Abstract

Extramammary Paget disease (EMPD) is a rare cutaneous neoplasm. Perianal Paget disease (PPD) is a subset of EMPD manifesting perianal lesions. Two cases of PPD in Severance Hospital are described in this article. A 65-year-old female and 78-year-old male patients visited our institution because of an unhealed perianal skin lesion despite treatment for a long period with topical agents. PPD was diagnosed by skin biopsies in both cases, and the patients underwent surgical treatment. Clinical manifestations, preoperative work-ups, and surgical treatments including different reconstruction methods are described in detail. As only sporadic PPD cases have been reported and no standard treatment has been established, we hope that our experience could contribute to improving the diagnosis and treatment of PPD patients.

Keyword

Extramammary Paget disease

MeSH Terms

Aged
Biopsy
Diagnosis
Female
Hope
Humans
Male
Paget Disease, Extramammary
Skin

Figure

• 

  Fig. 1 H&E and IHC stain in case 1. (A) Clusters of large epithelioid cells with pleomorphic nuclei and pale cytoplasm are present in the epidermis, consistent with EMPD (H&E, ×200). (B) Tumor cells show strong immunoreactivity for CK7 with cytoplasmic staining pattern (×100). (C) Tumor cells are negative for HMB-45 IHC, ruling out the possibility of melanoma (×100). CK7, cytokeratin 7; EMPD, extramammary Paget's disease; H&E, hematoxylin and eosin; HMB-45, human melanoma black-45; IHC, immunohistochemistry.

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  Fig. 2 Case 1. (A) Erythematous perianal skin lesion with slightly elevated patches, erosion, and brownish pigmentation; scout line determined by mapping biopsies (purple). (B) Wide excision with sphincter preservation. (C) Surgical specimen. (D, E) Internal pudendal artery perforator (IPAP) flap. (F) After completion of the IPAP flap and V-Y advancement flap surgery.

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  Fig. 3 H&E and IHC in case 2. (A) Clusters of large epithelioid cells with pleomorphic nuclei and pale cytoplasm in the epidermis (H&E, ×200). (B) Tumor cells showing strong immunoreactivity for CK7 (×100; red, in cytoplasm). (C) Tumor cells showing immunoreactivity for CEA (×100; brown, in cytoplasm). CEA, carcinoembryonic antigen; CK7, cytokeratin 7; H&E, hematoxylin and eosin; IHC, immunohistochemistry.

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  Fig. 4 Case 2. (A) Eroded macules with some whitish patches in the perianal area; scout line determined by mapping biopsies (purple). (B) Wide excision with sphincter preservation. (C, D) Skin graft surgery. (E) Healed wound.

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