EMPD Explanations and Presentations

The following EMPD clinical presentations and reference materials will give you a better understanding of extramammary Paget disease (EMPD). (Please be aware that the information shared in the links below may include images or other content that could be unsettling to some readers.)

PATIENTS’ EXPERIENCES WITH EXTRAMAMMARY PAGET DISEASE: AN ONLINE PILOT STUDY QUERYING A PATIENT SUPPORT GROUP

Extramammary Paget disease (EMPD) is a rare and lethal intraepithelial malignancy with poorly described treatment methodologies and outcomes. Incidence has been reported as 0.12 per 100,000 people, with an overall survival of 60% at 120 months post diagnosis. EMPD develops most frequently in the genital, perianal, and axillary regions, and commonly presents with symptoms of erythematous or pruritic rash or plaque. Patients often initially undergo rounds of ineffective treatments for a benign diagnosis before a correct diagnosis of EMPD is obtained. EMPD may present as poorly defined, multifocal, and subclinical lesions making the initial diagnosis challenging. Lesions may also become invasive, precluding definitive treatment. EMPD has also been associated with secondary internal malignancies, such as rectal, bladder,prostate, and endocervical cancers.

We developed an online survey to illustrate the heterogeneous care delivered to patients with EMPD by characterizing the clinical and pathologic characteristics of an international, online patient support group treated across different institutions.… Read More →

A primer on extramammary Paget’s disease for the urologist

Extramammary Paget’s disease (EMPD) is a rare and lethal intraepithelial malignancy that remains poorly understood. No standardized guidelines or consensus statements exist with regards to the diagnostic evaluation, therapeutic approaches and follow-up management. Complete surgical excision with negative margins has been accepted as the mainstay of treatment for EMPD to decrease the risk of local recurrence and to maximize durable cure.

Non-invasive therapies, such as laser therapy, photodynamic therapy, radiotherapy and topical chemotherapy have been utilized, but are best reserved for non-surgical candidates. While the debate on the surgical approach between wide local excision (WLE) and Mohs micrographic surgery (MMS) continues, several studies have demonstrated the ability of WLE to be performed safely and effectively and to yield equally satisfactory outcomes with similar rates of recurrence to MMS.

Patients undergoing surgical excision often require complex closures with skin grafting or local flaps to close genital defects. We aim to provide an up-to-date review of the current knowledge of EMPD. In addition to discussing the clinical presentation and prognostic outcomes, we focus and elaborate on the diagnostic approaches and treatment alternatives available. This information may serve as a primer for the urologist… Read More →

Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease

Extramammary Paget disease (EMPD) is a frequently recurring malignant neoplasm with metastatic potential that presents in older adults on the genital, perianal, and axillary skin. Extramammary Paget disease can precede or occur along with internal malignant neoplasms. A systematic review of the literature on EMPD from January 1990 to September 18, 2019, was conducted using MEDLINE, Embase, Web of Science Core Collection, and Cochrane Libraries. Analysis included 483 studies. A multidisciplinary expert panel evaluation of the findings led to the development of clinical care recommendations for EMPD.

The key findings were as follows: (1) Multiple skin biopsies, including those of any nodular areas, are critical for diagnosis. (2) Malignant neoplasm screening appropriate for age and anatomical site should be performed at baseline to distinguish between primary and secondary EMPD. (3) Routine use of sentinel lymph node biopsy or lymph node dissection is not recommended. (4) For intraepidermal EMPD, surgical and nonsurgical treatments may be used depending on patient and tumor characteristics, although cure rates may be superior with surgical approaches. For invasive EMPD, surgical resection with curative intent is preferred. (5) Patients with unresectable intraepidermal EMPD or patients who are medically unable to undergo surgery may receive nonsurgical treatments, including radiotherapy, imiquimod, photodynamic therapy, carbon dioxide laser therapy, or other modalities. (6) Distant metastatic disease may be treated with chemotherapy or individualized targeted approaches. (7) Close follow-up to monitor for recurrence is recommended for at least the first 5 years.

Clinical practice guidelines for EMPD provide guidance regarding recommended diagnostic approaches, differentiation between invasive and noninvasive disease, and use of surgical vs nonsurgical treatments. Prospective registries may further improve our understanding of the natural history of the disease in primary vs secondary EMPD, clarify features of high-risk tumors, and identify superior management approaches… Read More →

Extramammary Paget Disease

Extramammary Paget disease (EMPD) is a rare dermatologic condition that frequently presents in areas where apocrine sweat glands are abundant, most commonly the vulva, although perineal, scrotal, perianal, and penile skin may also be affected. Lesions clinically present as erythematous, well-demarcated plaques that may become erosive, ulcerated, scaly, or eczematous. Extramammary Paget disease has a female predominance and usually occurs in the sixth to eighth decades of life. Professionals disagree about many aspects of EMPD, for example, the prevalence of concurrent vulvar adenocarcinoma or invasive EMPD, association with regional and distant cancers, and recurrence rates following surgical excision. Early recognition is imperative because the diagnosis is frequently delayed and there is a high incidence of associated invasive disease.

The etiology of primary EMPD has been debated but appears to be of apocrine origin. Secondary extramammary Paget disease represents an extension of an underlying adnexal adenocarcinoma or an underlying visceral malignancy, most commonly urothelial or anorectal, but it may also be cervical, prostatic, ovarian, or endometrial… Read More →

Extramammary Paget disease

Extramammary Paget disease is an uncommon cancer characterised by a chronic eczema-like rash of the skin around the anogenital regions of males and females. Under the microscope extramammary Paget disease looks very similar to the more common type of mammary Paget disease that occurs on the breast.

Who gets extramammary Paget disease?

Extramammary Paget disease most commonly occurs in the vulva of women aged between 50–60 years. It can also affect males of similar age.… Read More →

Extramammary Paget's Disease - Causes, Symptoms, Diagnosis, and Treatment

Extramammary Paget’s disease (EMPD) is a rare type of slow-growing skin condition that might be associated with different types of cancer and usually causes a red, scaly, or crusty area of the skin. Sometimes, EMPD is left undiagnosed until many years after it starts to form.

EMPD is named after Sir James Paget, a famous 19th-century British physician. Several forms of cancer carry Paget’s name due to his work in the field of pathology. The term extramammary refers to the outside of the breast and helps prevent confusion with other diseases that are also named for Paget (such as Paget disease of the breast).

About 7 to 40 percent of cases of EMPD have been associated with underlying cancer. … Read More →

additional EMPD information

Extramammary Paget’s disease (EMPD) symptoms, treatment, and overview information may be found here:

The content presented on this website, encompassing both textual material and visual media, serves educational and informational purposes aimed at aiding individuals affected by extramammary Paget’s disease (EMPD) and their families in comprehending the complexities associated with EMPD. It is imperative to note that the website is not designed to serve as a replacement for professional medical advice, diagnosis, or treatment. Furthermore, the website does not advocate for or endorse any specific treatments, tests, physicians, procedures, or products. Should you suspect that you are experiencing symptoms of EMPD, we strongly advise you to seek guidance from your healthcare provider.