Extramammary Paget Disease Clinical Presentation

  • Author: Neil Sandhu, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Oct 20, 2011
 

History

The possibility of extramammary Paget disease (EMPD) should be carefully considered in any patient with chronic dermatitis of the groin, vulva, or perianal area. Patients with EMPD usually present with nonresolving eczematous lesions in the groin, genitalia, perineum, or perianal area.[3] The most common symptom of EMPD is intense pruritus; most patients have only pruritus in the affected area and no other symptoms. Pain and bleeding may occur in longer-standing lesions.

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Physical Examination

At clinical examination, EMPD may appear as chronic intertrigo or presumed tinea cruris. It may appear eczematous, and it has usually been present for a long time before biopsy is performed to confirm the diagnosis. The genitalia, perineum, axillae, and external auditory canal are rich in apocrine glands; therefore, these are the usual sites of EMPD involvement.

Early skin changes may be subtle and vary according to location. Initially, only slight erythema, crusting, and increased maceration may be noted. Pruritus commonly leads to prominent excoriations and lichenification. Lesional progression leads to a unilateral sharply marginated plaque with distinct erythema. Superficial erosion or scaling may develop in mature lesions.

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Contributor Information and Disclosures
Author

Neil Sandhu, MD  Resident Physician, Department of Dermatology, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Neil Sandhu, MD is a member of the following medical societies: American Dermatological Association, American Medical Association, American Medical Student Association/Foundation, American Roentgen Ray Society, American Society of Dermatology, and Radiological Society of North America

Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Marjan Garmyn, MD, PhD Professor, Faculty of Medicine, Katholieke Universiteit Leuven, Belgium; Chair and Adjunct Head, Department of Dermatology, University of Leuven, Belgium

Disclosure: Nothing to disclose.

Michael J Wells, MD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Joseph L Wilde, MD Chief, Department of Dermatology, Vicenza Army Health Center, Italy

Joseph L Wilde, MD is a member of the following medical societies: American College of Mohs Micrographic Surgery and Cutaneous Oncology

Disclosure: Nothing to disclose.

References

  1. Roy J, Mirnezami A, Gatt M, Sasapu K, Scott N, Sagar P. A rare case of Paget's disease in a retrorectal dermoid cyst. Colorectal Dis. Nov 2 2009;[Medline].

  2. Chanda JJ. Extramammary Paget's disease: prognosis and relationship to internal malignancy. J Am Acad Dermatol. Dec 1985;13(6):1009-14. [Medline].

  3. Bagby CM, MacLennan GT. Extramammary Paget's disease of the penis and scrotum. J Urol. Dec 2009;182(6):2908-9. [Medline].

  4. Cho SB, Yun M, Lee MG, Chung KY. Variable patterns of positron emission tomography in the assessment of patients with extramammary Paget's disease. J Am Acad Dermatol. Feb 2005;52(2):353-5. [Medline].

  5. D'Agostino M, Cinelli C, Willard R, Hofmann J, Jellinek N, Robinson-Bostom L. Epidermotropic Merkel cell carcinoma: a case series with histopathologic examination. J Am Acad Dermatol. Mar 2010;62(3):463-8. [Medline].

  6. Perrotto J, Abbott JJ, Ceilley RI, Ahmed I. The Role of Immunohistochemistry in Discriminating Primary From Secondary Extramammary Paget Disease. Am J Dermatopathol. Jan 4 2010;[Medline].

  7. Beleznay KM, Levesque MA, Gill S. Response to 5-fluorouracil in metastatic extramammary Paget disease of the scrotum presenting as pancytopenia and back pain. Curr Oncol. Sep 2009;16(5):81-3. [Medline].

  8. Sendagorta E, Herranz P, Feito M, et al. Successful treatment of three cases of primary extramammary Paget's disease of the vulva with Imiquimod - proposal of a therapeutic schedule. J Eur Acad Dermatol Venereol. Oct 15 2009;[Medline].

  9. Takahagi S, Noda H, Kamegashira A, et al. Metastatic extramammary Paget's disease treated with paclitaxel and trastuzumab combination chemotherapy. J Dermatol. Aug 2009;36(8):457-61. [Medline].

  10. Cecchi R, Pavesi M, Bartoli L, Brunetti L, Rapicano V. Perineal extramammary Paget disease responsive to topical imiquimod. J Dtsch Dermatol Ges. Jan 2010;8(1):38-40. [Medline].

  11. Appert DL, Otley CC, Phillips PK, Roenigk RK. Role of multiple scouting biopsies before Mohs micrographic surgery for extramammary Paget's disease. Dermatol Surg. Nov 2005;31(11 Pt 1):1417-22. [Medline].

  12. Coldiron BM, Goldsmith BA, Robinson JK. Surgical treatment of extramammary Paget's disease. A report of six cases and a reexamination of Mohs micrographic surgery compared with conventional surgical excision. Cancer. Feb 15 1991;67(4):933-8. [Medline].

  13. Hendi A, Brodland DG, Zitelli JA. Extramammary Paget's disease: surgical treatment with Mohs micrographic surgery. J Am Acad Dermatol. Nov 2004;51(5):767-73. [Medline].

 
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