Extramammary Paget Disease (Paget Disease, Extramammary)

A rare cutaneous neoplasm that occurs in the elderly. It develops more frequently in women and predominantly involves apocrine gland-bearing areas, especially the vulva, scrotum, and perianal areas. The lesions develop as erythematous scaly patches that progress to crusted, pruritic, erythematous plaques. The clinical differential diagnosis includes squamous cell carcinoma in situ and superficial fungal infection. It is generally thought to be an adenocarcinoma of the epidermis, from which it extends into the contiguous epithelium of hair follicles and eccrine sweat ducts. (DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1478)
Also Known As:
Paget Disease, Extramammary; Extra-Mammary Paget Disease; Extra-Mammary Paget's Disease; Extramammary Paget's Disease; Paget Disease, Extra-Mammary; Paget's Disease, Extra-Mammary; Extra Mammary Paget Disease; Extra Mammary Paget's Disease; Extra-Mammary Pagets Disease; Extramammary Pagets Disease; Extramammary, Paget Disease; Paget Disease, Extra Mammary; Paget's Disease, Extra Mammary; Pagets Disease, Extra-Mammary; Pagets Disease, Extramammary; Paget Disease Extramammary; Paget's Disease, Extramammary
Networked: 279 relevant articles (19 outcomes, 20 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Carcinoma (Carcinomatosis)
2. Melanoma (Melanoma, Malignant)
3. Squamous Cell Carcinoma (Epidermoid Carcinoma)
4. Basal Cell Carcinoma (Rodent Ulcer)
5. Neoplasms (Cancer)


1. Furue, Masutaka: 9 articles (10/2011 - 01/2007)
2. Moroi, Yoichi: 8 articles (10/2011 - 01/2007)
3. Takeuchi, Satoshi: 8 articles (10/2011 - 01/2007)
4. Xie, Lining: 5 articles (10/2011 - 03/2010)
5. Nakahara, Takeshi: 5 articles (10/2011 - 01/2007)
6. Takahara, Masakazu: 5 articles (10/2011 - 03/2007)
7. Hayashida, Sayaka: 5 articles (07/2011 - 06/2008)
8. Uchi, Hiroshi: 5 articles (05/2010 - 01/2007)
9. Tu, Ya-Ting: 4 articles (10/2011 - 06/2008)
10. Tsuji, Gaku: 4 articles (10/2011 - 03/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Extramammary Paget Disease:
1. imiquimod (Aldara)FDA LinkGeneric
2. docetaxel (Taxotere)FDA Link
3. 1-phenyl-3,3-dimethyltriazene (PDT)IBA
4. Dermatofibrosarcoma protuberansIBA
03/01/2007 - "Retrospective chart review of 26,000 cases treated with MMS at the Geisinger Medical Center Department of Dermatology during a 16-year period with the following diagnoses: poorly differentiated squamous cell carcinoma (PDSCC), dermatofibrosarcoma protuberans (DFSP), microcystic adnexal carcinoma (MAC), extramammary Paget's disease (EMPD), Merkel cell carcinoma (MCC), and sebaceous carcinoma (SEB CA). "
12/01/1996 - "We describe our experience of this method in the management of squamous cell carcinomas (11), extramammary Paget's disease (two), microcystic adnexal cell carcinomas (two), dermatofibrosarcoma protuberans (two), and primary cutaneous neuroendocrine carcinoma (Merkel cell carcinoma) (one). "
08/01/2005 - "Its efficacy in treating BCC and SCC has led clinicians to explore the role of Mohs micrographic surgery in the management of less common cutaneous neoplasms, such as melanoma, Merkel cell carcinoma, dermatofibrosarcoma protuberans, extramammary Paget's disease, and microcystic adnexal carcinoma."
12/01/2008 - "In recent years, the application of immunostaining techniques has facilitated the successful removal of a number of common and less common cutaneous malignancies, including basal cell carcinoma, squamous cell carcinoma, malignant melanoma, dermatofibrosarcoma protuberans, microcystic adnexal carcinoma, sebaceous carcinoma, atypical fibroxanthoma, extramammary Paget's disease, and even sarcomas. "
08/01/1990 - "Between 1970 and 1984 in Rochester, Minnesota, rare skin cancers developed in 15 local residents: cutaneous T cell lymphoma (six subjects), dermatofibrosarcoma protuberans (four), adenocarcinoma of sweat glands (two), Merkel cell carcinoma (one), liposarcoma (one), and extramammary Paget's disease (one). "
5. Cystic Fibrosis Transmembrane Conductance Regulator (Protein, CFTR)IBA
6. MucinsIBA
7. Keratins (Keratin)IBA
8. Proteins (Proteins, Gene)IBA
9. Carcinoembryonic AntigenIBA
10. AntigensIBA

Therapies and Procedures

1. Radiotherapy
2. Drug Therapy (Chemotherapy)
3. Mohs Surgery (Mohs' Micrographic Surgery)
4. Photochemotherapy (Photodynamic Therapy)
5. Neoadjuvant Therapy