Extramammary Paget's disease

From WikiMD.com - Food, Medicine & Wellness Encyclopedia

Dr.Prab.jpg

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

Extramammary Paget's disease
Histopathology of extramammary Paget's disease
Synonyms EMPD
Pronounce N/A
Specialty N/A
Symptoms Erythematous plaques, pruritus, burning sensation
Complications Invasive carcinoma, metastasis
Onset Typically in elderly individuals
Duration Chronic
Types N/A
Causes Unknown, possibly related to apocrine glands
Risks Age, genetic predisposition, immunosuppression
Diagnosis Biopsy, histopathology
Differential diagnosis Eczema, psoriasis, seborrheic dermatitis
Prevention N/A
Treatment Surgical excision, radiation therapy, topical chemotherapy
Medication N/A
Prognosis Variable, depends on stage and treatment
Frequency Rare
Deaths N/A


Extramammary Paget's disease (EMPD) is a rare, slow-growing malignancy that typically affects areas of the body with apocrine glands, such as the genital, perianal, and axillary regions. It is similar in appearance and pathology to Paget's disease of the breast, but occurs outside the mammary glands.

Presentation[edit | edit source]

EMPD often presents as a red, scaly, and itchy rash that can be mistaken for benign skin conditions such as eczema or dermatitis. The affected area may also exhibit ulceration, bleeding, or a white plaque-like appearance. Due to its nonspecific symptoms, EMPD is frequently misdiagnosed, leading to delays in appropriate treatment.

Pathophysiology[edit | edit source]

The exact cause of EMPD is not well understood. It is believed to originate from the apocrine gland cells or from underlying malignancies such as adenocarcinoma. The disease can be classified into primary and secondary types. Primary EMPD arises de novo in the epidermis, while secondary EMPD is associated with an underlying malignancy.

Diagnosis[edit | edit source]

Diagnosis of EMPD is confirmed through a skin biopsy, which reveals characteristic Paget cells—large cells with abundant pale cytoplasm and prominent nuclei. Immunohistochemical staining is often used to differentiate EMPD from other skin conditions. Common markers include cytokeratin 7 (CK7), carcinoembryonic antigen (CEA), and gross cystic disease fluid protein-15 (GCDFP-15).

Treatment[edit | edit source]

Treatment options for EMPD include surgical excision, Mohs micrographic surgery, and topical therapies such as imiquimod or 5-fluorouracil. In cases where surgery is not feasible, radiation therapy or systemic chemotherapy may be considered. The choice of treatment depends on the extent of the disease and the presence of any underlying malignancies.

Prognosis[edit | edit source]

The prognosis for patients with EMPD varies. Localized disease has a better prognosis, while cases associated with underlying malignancies or metastasis have a poorer outcome. Regular follow-up is essential to monitor for recurrence or the development of secondary malignancies.

See also[edit | edit source]

Stub icon
   This article is a medical stub. You can help WikiMD by expanding it!



WikiMD
Navigation: Wellness - Encyclopedia - Health topics - Disease Index‏‎ - Drugs - World Directory - Gray's Anatomy - Keto diet - Recipes

Transform your life with W8MD's budget GLP1 injections in NYC from $125 and up biweekly

W8mdlogo.png
W8MD weight loss doctors team

W8MD offers a medical weight loss program NYC and a clinic to lose weight in Philadelphia. Our W8MD's physician supervised medical weight loss centers in NYC provides expert medical guidance, and offers telemedicine options for convenience.

Why choose W8MD?

Book Your Appointment

Start your NYC weight loss journey today at our NYC medical weight loss, and Philadelphia and visit Philadelphia medical weight loss Call (718)946-5500 for NY and 215 676 2334 for PA

Search WikiMD

Ad.Tired of being Overweight? Try W8MD's NYC physician weight loss.
Semaglutide (Ozempic / Wegovy and Tirzepatide (Mounjaro / Zepbound) available. Call 718 946 5500.
Advertise on WikiMD

WikiMD's Wellness Encyclopedia

Let Food Be Thy Medicine
Medicine Thy Food - Hippocrates

Medical Disclaimer: WikiMD is not a substitute for professional medical advice. The information on WikiMD is provided as an information resource only, may be incorrect, outdated or misleading, and is not to be used or relied on for any diagnostic or treatment purposes. Please consult your health care provider before making any healthcare decisions or for guidance about a specific medical condition. WikiMD expressly disclaims responsibility, and shall have no liability, for any damages, loss, injury, or liability whatsoever suffered as a result of your reliance on the information contained in this site. By visiting this site you agree to the foregoing terms and conditions, which may from time to time be changed or supplemented by WikiMD. If you do not agree to the foregoing terms and conditions, you should not enter or use this site. See full disclaimer.
Credits:Most images are courtesy of Wikimedia commons, and templates, categories Wikipedia, licensed under CC BY SA or similar.

Contributors: Prab R. Tumpati, MD