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 that is recalcitrant to treatment. Patients with EMPD usually present with nonresolving eczematous lesions in the groin, genitalia, perineum, or perianal area.^[16]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. Burning, tenderness, and edema may also be experienced over the area of involvement.

Physical Examination

At clinical examination, extramammary Paget disease (EMPD) may appear as chronic intertrigo, eczematous dermatitis, or presumed tinea cruris. Lesions can be nonspecific, making diagnosis challenging. EMPD usually has been present for several years before biopsy is performed to confirm the diagnosis, with a reported median delay of 2 years before proper diagnosis.^[1]The genitalia, perineum, axillae, and external auditory canal are rich in apocrine glands; therefore, these are the usual sites of EMPD involvement, which is often multifocal. In males, the penoscrotal and perianal areas are most frequently involved, as is the vulva in women.^[1]Rarely, it may occur in the buttock, knee, glans penis, thigh, abdomen, umbilicus, lower anterior chest, and scalp.^[17]See the image below.

Classic appearance of extramammary Paget disease (EMPD). Courtesy of Kenneth E Greer, MD.

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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. Scattered areas of erosion and white scale can result in a characteristic "strawberries and cream" appearance (see the image below).^[1]Lesional progression leads to a unilateral sharply marginated plaque with distinct erythema. Hypopigmentation or hyperpigmentation occurs, and small pale islands are often observed over the lesion.

Classic "strawberries and cream" appearance of extramammary Paget disease (EMPD). Courtesy of Kenneth E Greer, MD.

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Hard nodules and regional lymph node enlargement may develop in cases with an underlying carcinoma. "Underpants-pattern erythema" is described in cases of genital EMPD as a result of metastases to the lymphatic system. This pattern starts in the groin and spreads peripherally to areas typically covered by the underwear. This portends a grim prognosis owing to the possibility of rapidly fatal distant metastases.^[1]

Differential Diagnoses

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Ohara K, Fujisawa Y, Yoshino K, Kiyohara Y, Kadono T, Murata Y, et al. A proposal for a TNM staging system for extramammary Paget disease: Retrospective analysis of 301 patients with invasive primary tumors. J Dermatol Sci. 2016 Sep. 83 (3):234-9. [QxMD MEDLINE Link].
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Kaku-Ito Y, Ito T, Tsuji G, Nakahara T, Hagihara A, Furue M, et al. Evaluation of mapping biopsies for extramammary Paget disease: A retrospective study. J Am Acad Dermatol. 2018 Jun. 78 (6):1171-1177.e4. [QxMD MEDLINE Link].
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Wang Z, Lu M, Dong GQ, Jiang YQ, Lin MS, Cai ZK, et al. Penile and scrotal Paget's disease: 130 Chinese patients with long-term follow-up. BJU Int. 2008 Aug. 102 (4):485-8. [QxMD MEDLINE Link].
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Damavandy AA, Terushkin V, Zitelli JA, Brodland DG, Miller CJ, Etzkorn JR, et al. Intraoperative Immunostaining for Cytokeratin-7 During Mohs Micrographic Surgery Demonstrates Low Local Recurrence Rates in Extramammary Paget's Disease. Dermatol Surg. 2018 Mar. 44 (3):354-364. [QxMD MEDLINE Link].
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Media Gallery

Classic histology of extramammary Paget disease (EMPD). Clusters of large cells with pale, vacuolated cytoplasm scattered throughout the epidermis.
High power image of extramammary Paget disease (EMPD). Clusters of large cells with pale, vacuolated cytoplasm scattered throughout the epidermis.
Classic appearance of extramammary Paget disease (EMPD). Courtesy of Kenneth E Greer, MD.
Classic "strawberries and cream" appearance of extramammary Paget disease (EMPD). Courtesy of Kenneth E Greer, MD.

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Author

Richard Harold "Hal" Flowers, IV, MD Assistant Professor, Department of Dermatology, University of Virginia Health System

Richard Harold "Hal" Flowers, IV, MD is a member of the following medical societies: American Academy of Dermatology, Medical Dermatology Society, Rheumatologic Dermatology Society

Disclosure: Nothing to disclose.

Coauthor(s)

Stephany Vittitow, BA MD Candidate, University of Virginia School of Medicine

Stephany Vittitow, BA is a member of the following medical societies: American Medical Association, American Medical Women's Association, National First Generation and Low-income in Medicine Association

Disclosure: Nothing to disclose.

Seth M Martin, MD Resident Physician, Department of Dermatology, UVA Health, University of Virginia School of Medicine

Seth M Martin, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Gold Humanism Honor Society, Medical Dermatology Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Neil Sandhu, MD, FAAD Dermatologist (Medical/Cosmetic) and Mohs Surgeon, Gulf Coast Dermatology

Neil Sandhu, MD, FAAD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery

Disclosure: Nothing to disclose.

Blanca Anais Estupiñan University of Central Florida College of Medicine

Blanca Anais Estupiñan is a member of the following medical societies: American College of Physicians, American Medical Association, American Psychiatric Association

Disclosure: Nothing to disclose.

Acknowledgements

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.