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Extramammary Paget Disease: A Review of the Literature-Part I: History, Epidemiology, Pathogenesis, Presentation, Histopathology, and Diagnostic Work-up.

Abstract

BACKGROUND

Extramammary Paget disease (EMPD) is a rare malignancy with unclear pathophysiology that occurs predominantly on apocrine rich skin. Surgery is the treatment of choice; however, procedures tend to be extensive and associated with a high rate of recurrence.

OBJECTIVE

To review the current literature on EMPD regarding epidemiology, pathogenesis, clinical presentation, histology, diagnostic work-up, treatment, and prognosis.

MATERIALS AND METHODS

Literature review using PubMed search for articles related to EMPD.

RESULTS

Extramammary Paget disease classically presents as a slowly growing red plaque, which often mimics an inflammatory condition leading to significant delay in diagnosis. Diagnosis requires histopathologic examination and is often supported by immunohistochemical analysis. Once a diagnosis of EMPD is made, the patient must be risk-stratified and evaluated for an underlying malignancy.

CONCLUSION

Standard of treatment is surgery, with data suggesting that Mohs micrographic surgery may have superior clinical outcomes and lower recurrence rates. Alternatives such as photodynamic therapy and topicals have been explored and may be appropriate in certain situations. Patients with EMPD generally have a good prognosis with a 5-year overall survival rate of 75% to 95%.

Authors+Show Affiliations

*Both authors are affiliated with the Department of Internal Medicine, Division of Dermatology, Washington University, St. Louis, Missouri.Both authors are affiliated with the Department of Internal Medicine, Division of Dermatology, Washington University, St. Louis, Missouri.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31356440

Citation

Morris, Caroline R., and Eva A. Hurst. "Extramammary Paget Disease: a Review of the Literature-Part I: History, Epidemiology, Pathogenesis, Presentation, Histopathology, and Diagnostic Work-up." Dermatologic Surgery : Official Publication for American Society for Dermatologic Surgery [et Al.], 2019.
Morris CR, Hurst EA. Extramammary Paget Disease: A Review of the Literature-Part I: History, Epidemiology, Pathogenesis, Presentation, Histopathology, and Diagnostic Work-up. Dermatol Surg. 2019.
Morris, C. R., & Hurst, E. A. (2019). Extramammary Paget Disease: A Review of the Literature-Part I: History, Epidemiology, Pathogenesis, Presentation, Histopathology, and Diagnostic Work-up. Dermatologic Surgery : Official Publication for American Society for Dermatologic Surgery [et Al.], doi:10.1097/DSS.0000000000002064.
Morris CR, Hurst EA. Extramammary Paget Disease: a Review of the Literature-Part I: History, Epidemiology, Pathogenesis, Presentation, Histopathology, and Diagnostic Work-up. Dermatol Surg. 2019 Jul 24; PubMed PMID: 31356440.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Extramammary Paget Disease: A Review of the Literature-Part I: History, Epidemiology, Pathogenesis, Presentation, Histopathology, and Diagnostic Work-up. AU - Morris,Caroline R, AU - Hurst,Eva A, Y1 - 2019/07/24/ PY - 2019/7/30/entrez PY - 2019/7/30/pubmed PY - 2019/7/30/medline JF - Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.] JO - Dermatol Surg N2 - BACKGROUND: Extramammary Paget disease (EMPD) is a rare malignancy with unclear pathophysiology that occurs predominantly on apocrine rich skin. Surgery is the treatment of choice; however, procedures tend to be extensive and associated with a high rate of recurrence. OBJECTIVE: To review the current literature on EMPD regarding epidemiology, pathogenesis, clinical presentation, histology, diagnostic work-up, treatment, and prognosis. MATERIALS AND METHODS: Literature review using PubMed search for articles related to EMPD. RESULTS: Extramammary Paget disease classically presents as a slowly growing red plaque, which often mimics an inflammatory condition leading to significant delay in diagnosis. Diagnosis requires histopathologic examination and is often supported by immunohistochemical analysis. Once a diagnosis of EMPD is made, the patient must be risk-stratified and evaluated for an underlying malignancy. CONCLUSION: Standard of treatment is surgery, with data suggesting that Mohs micrographic surgery may have superior clinical outcomes and lower recurrence rates. Alternatives such as photodynamic therapy and topicals have been explored and may be appropriate in certain situations. Patients with EMPD generally have a good prognosis with a 5-year overall survival rate of 75% to 95%. SN - 1524-4725 UR - https://www.unboundmedicine.com/medline/citation/31356440/Extramammary_Paget_Disease:_A_Review_of_the_Literature_Part_I:_History_Epidemiology_Pathogenesis_Presentation_Histopathology_and_Diagnostic_Work_up_ L2 - http://dx.doi.org/10.1097/DSS.0000000000002064 DB - PRIME DP - Unbound Medicine ER -