J Breast Cancer.  2008 Sep;11(3):156-159. 10.4048/jbc.2008.11.3.156.

A Case of Extramammary Paget's Disease of the Axilla

• Youn HJ
• Jung SH
• Kim JC

• KMID: 1485110
• DOI: http://doi.org/10.4048/jbc.2008.11.3.156

Abstract

Extramammary Paget's disease is an uncommon intraepithelial malignancy that usually presents as a well circumscribed, reddish eczematous patch on the apocrine-bearing regions such as the genital and perineal areas. It progresses slowly with local extension. However, it may become invasive and may be associated with internal malignancies. Extramammary Paget's disease has high local recurrence rates after complete surgical excision and therefore, long-term follow-up is necessary. Extramammary Paget's disease of the axilla is extremely rare, with few reported cases. We report here a case of extramammary Paget's disease of the axilla in a 68-yr-old man, with a review of the literature.

Keyword

Axilla; Extramammary Paget's disease

MeSH Terms

Axilla
Follow-Up Studies
Paget Disease, Extramammary
Recurrence

Figure

• 

  Fig 1 A 68-yr-old man presented with a 3×1.5 cm sized, well circumscribed, reddish erosive and eczematous patch on the right axilla.

• 

  Fig 2 Microscopic and immunohistochemical findings of extramammary Paget's disease. (A) Paget cells are arranged singly or in small groups in the basal and parabasal regions of the epidermis. Paget cells have abundant cytoplasm and large pleomorphic nuclei. Occasional cells have an eccentric nucleus and atypical mitosis is present (H&E stain, ×200). (B) Paget cells show positive staining for cytokeratin 7 (H&E stain, ×200).

Reference

1. Lloyd J, Flanagan A. Mammary and extramammary Paget's disease. J Clin Pathol. 2000. 53:742–749.

2. Kanitakis J. Mammary and extramammary Paget's disease. J Eur Acad Dermatol Venereol. 2007. 21:581–590.

3. Fardal R, Kierland R, Clagett O, Woolner L. Prognosis in cutaneous Paget's disease. Postgrad Med. 1967. 36:584–593.

4. Ohara K, Onishi Y, Jawabata Y. Diagnosis and treatment of extramammary Paget's disease. Skin Cancer. 1993. 8:187–208.

5. Chanda JJ. Extramammary Paget's disease: prognosis and relationship to internal malignancy. J Am Acad Dermatol. 1985. 13:1009–1014.

6. Sitakalin C, Ackerman AB. Mammary and extramammary Paget's disease. Am J Dermatopathol. 1985. 7:335–340.

7. Jones RE Jr, Austin C, Ackerman AB. Extramammary Paget's disease. A critical reexamination. Am J Dermatopathol. 1979. 1:101–132.

8. Zollo JD, Zeitouni NC. The Roswell Park Cancer Institute experience with extramammary Paget's disease. Br J Dermatol. 2000. 142:59–65.

9. Murata Y, Kumano K. Extramammary Paget's disease of the genitalia with clinically clear margins can be adequately resected with 1 cm margin. Eur J Dermatol. 2005. 15:168–170.

10. Choe SW, Kim JE, Seo SJ, Kim MN, Hong CK, Ro BI. A case of extramammary Paget's disease of the axilla. Korean J Dermatol. 2004. 42:624–626.

11. Byun HJ, Yu DK, Cho KH. A case of extramammary Paget's disease of the axilla. Korean J Dermatol. 2006. 44:1013–1015.

12. Crocker HR. Paget's disease affecting the scrotum and penis. Trans Pathol Soc London. 1889. 40:187–191. Cited from Kanitakis J. Mammary and extramammary Paget's disease. J Eur Acad Dermatol Venereol 2007;21:581-90.

13. Urabe A, Matsukuma A, Shimizu N, Nishimura M, Wala H, Hori Y. Extramammary Paget's disease: comparative histopathologic studies of intraductal carcinoma of the breast and apocrine adenocarcinoma. J Cutan Pathol. 1990. 17:257–265.

14. Kim YJ, Kim MY, Kang H, Park CJ, Park YM, Kim HO. Clinical study of 28 cases of extramammary Paget's disease. Korean J Dermatol. 2005. 43:1321–1325.

15. Son IY, Sung HT, Chun HS, Han J, Lee ES. A case of extramammary Paget's disease on the scalp. Ann Dermatol. 1999. 11:189–192.

16. Gonzalez-Castro J, Iranzo P, Palou J, Mascaro JM. Extramammary Paget's disease involving the external ear. Br J Dermatol. 1998. 138:904–927.

17. Onish Y, Ohara K. Ectopic extramammary Paget's disease affecting the upper abdomen. Br J Dermatol. 1996. 134:958–961.

18. Kawatsu T, Miki Y. Triple extramammary Paget's disease. Arch Dermatol. 1971. 104:316–319.

19. Kitajima S, Yamamoto K, Tsui T, Shwartz RA. Triple extramammary Paget's disease. Dermatol Surg. 1997. 23:1035–1038.

20. Ohnishi T, Watanbe S. The use of cytokeratins 7 and 20 in the diagnosis of primary and secondary extramammary Paget's disease. Br J Dermatol. 2000. 142:243–247.

21. Connolly SM. Freeberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, editors. Mammary and extramammary Paget's disease. Dermatology in general medicine. 2003. 6th ed. New York: McGraw-Hill;813–817.

22. Chang YT, Liu HN, Wong CK. Extramammary Paget's disease: a report of 22 cases in Chineses males. J Dermatol. 1996. 23:320–324.

23. Murata Y, Kumano K. Extramammary Paget's disease of the genitalia with clinically clear margins can be adequately resected with 1 cm margin. Eur J Dermatol. 2005. 15:168–170.

24. Hendi A, Brodland DG, Zitelli JA. Extramammary Paget's disease: surgical treatment with Mohs micrographic surgery. J Am Acad Dermatol. 2004. 51:767–773.

25. Tsutsumida A, Yamamoto Y, Minakawa H, Yoshida T, Kokubu I, Sugihara T. Indications for lymph node dissection in the treatment of extramammary Paget's disease. Dermatol Surg. 2003. 29:21–24.

26. Cohen PR, Schulze KE, Tschen JA, Hetherington GW, Nelson BR. Treatment of extramammary Paget disease with topical imiquimod cream: case report and literature review. South Med J. 2006. 99:396–402.

27. Chiu TW, Wong PS, Ahmed K, Lam SC, Ying SY, Burd A. Extramammary Paget's disease in Chinese males: a 21-year experience. World J Surg. 2007. 31:1941–1946.