Have you recently been DIAGNOSED with extramammary Paget's disease (EMPD)?

Receiving a diagnosis of extramammary Paget’s disease (EMPD) can be overwhelming and disconcerting, both for the individual affected and their loved ones. This guide aims to provide clarity and understanding amidst the uncertainty that accompanies such a diagnosis. Extramammary Paget’s disease is a rare form of cancer that presents unique challenges in terms of symptoms, diagnosis, and treatment. Throughout this resource, we endeavor to shed light on the intricacies of EMPD, offering insights into its symptoms, the diagnostic process, and the various treatment options available. Whether you are grappling with this diagnosis firsthand or supporting a family member through their journey, our aim is to equip you with the knowledge and resources necessary to navigate the complexities of extramammary Paget’s disease (EMPD).

EMPD Overview

Extramammary Paget's disease (EMPD) is a rare form of cancer primarily affecting both men and women's skin. It is characterized by the presence of abnormal cancer cells, generally within the skin's epidermis or dermis. Unlike typical skin cancers triggered by ultraviolet radiation exposure, EMPD usually develops in areas housing apocrine sweat glands. The condition often presents as red, scaly patches or plaques on the skin, sometimes resembling other dermatological conditions. While the genital and perianal regions are the most common sites for EMPD, it can also occur in areas such as the axilla (armpit), groin, buttocks, thighs, and abdomen. In rarer instances, EMPD may appear in locations like the arm, neck, and head. Read More →

EMPD Symptoms

Symptoms of extramammary Paget's disease (EMPD) vary widely among patients due to the location, depth, and overall extent of the disease. Common symptoms include rash, persistent itching, burning sensation, pain, soreness, pruritus, scaliness, crustiness, inflammation, irritation, bleeding lesions, subtle skin changes, and enlarged regional lymph nodes. EMPD is sometimes confused with jock-itch. Although EMPD often progresses slowly, if left untreated, it can metastasize. Read More →

EMPD Diagnosis

Diagnosing extramammary Paget's disease (EMPD) can be challenging due to its resemblance to other conditions, leading to delayed diagnosis. Moreover, many physicians lack firsthand experience with EMPD cases. While a visual inspection may raise suspicions, a definitive diagnosis requires biopsy often accompanied by immunohistochemical staining. EMPD diagnoses may be categorized as either EMPD or invasive EMPD, and further classified as Primary EMPD (cutaneous origin) or Secondary EMPD (non-cutaneous origin), with classifications or staging varying across the world. The depth of invasion is a critical factor influencing EMPD's progression.

EMPD Treatment

Treatment for extramammary Paget's disease (EMPD) varies widely depending on factors such as disease location, depth, geographical location of the patient, and physician expertise. Unlike many cancers, EMPD lacks standardized treatment guidelines, prompting many patients to seek care from specialized or university medical centers. Treatment options may include surgery (wide local excision or Mohs), topical therapies, radiation, laser ablation, photodynamic therapy, chemotherapy, or immunotherapy. Each treatment modality carries potential side effects, emphasizing the importance of collaborative decision-making with healthcare providers. Many EMPD patients, and their doctors, have found the EMPD study “Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease” to be useful in understanding EMPD treatment options.

Elder Disease

While the average age of extramammary Paget's disease (EMPD) diagnosis is 66, cases have been reported in individuals as young as 17. Vigilance for EMPD symptoms or diagnosis is essential for all adults.

Detecting EMPD

Following an extramammary Paget's disease (EMPD) diagnosis, assessing the cancer's extent can be challenging. Physicians may use various techniques such as scouting or mapping biopsies or in vivo reflectance confocal microscopy (RCM) to determine the disease's spread. Additional diagnostic tools may include full skin examinations, lymph node palpation, ultrasound, computed tomography (CT) scans, positron emission tomography (PET) scans, sigmoidoscopy, colonoscopy, endoscopy, cystoscopy, and blood tests like carcinoembryonic antigen (CEA) screening. Female patients may undergo Papanicolaou smears and mammography, while male patients may consider PSA blood tests. However, specific tests may vary.

EMPD Rarity

While rare, the incidence of extramammary Paget's disease (EMPD) worldwide appears to be increasing annually, with misdiagnosis stemming from symptom similarities with other conditions.

Familial Connection

Although uncommon, familial cases of extramammary Paget's disease (EMPD) have been documented, involving relatives such as parents, children, or siblings.

Psychological Impacts

In addition to physical effects, some extramammary Paget's disease (EMPD) patients experience psychological distress, including anxiety, depression and post-traumatic stress disorder (PTSD), highlighting the need for holistic care.

EMPD Confusion

Extramammary Paget's disease (EMPD) is often mistaken for other Paget diseases. First diagnosed by Sir James Paget in 1874, EMPD shares cellular similarities with conditions like Mammary Paget's Disease (MPD). However, their primary distinction lies in their anatomical locations (the areola or nipple versus the genital, anal or other areas). Paget's disease of bone is unrelated to EMPD or MPD, with its etiology suspected to involve environmental or other genetic factors.

EMPD Support Group

For individuals and families affected by extramammary Paget's disease (EMPD), a support group is available. Additional information can be accessed here:

Want to learn more About EMPD?

If you are looking for additional information on extramammary Paget's disease (EMPD) you may want to check out the many sections on this website including the Frequently Asked Questions (FAQ) or the EMPD symptoms pages.

This information was written by EMPD patients and family members and is believed to be accurate at the time of publication. 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.