What are the other Names for this Condition? (Also known as/Synonyms)

• Secondary Cutaneous EMPD
• Secondary Cutaneous Extramammary Paget Disease
• Skin Involvement in Extramammary Paget’s Disease

What is Secondary Cutaneous Extramammary Paget’s Disease? (Definition/Background Information)

• Mammary Paget disease typically arises in the breast skin surface in females; mostly around the nipple and areola. It is almost always associated with an underlying breast cancer. Paget’s disease outside of the breast is rare. Such cases are termed extramammary Paget’s disease (EMPD). Both males and females may be affected
• Secondary Cutaneous Extramammary Paget Disease is the term used when Paget’s Disease develops secondary to systemic disease; meaning that the EMPD affects internal organs first, before affecting the skin. In a majority, the sites involved are in the abdominal-pelvic region (the urinary bladder, large intestine, and rectum)
• Even though no specific risk factors are identified; in general, Paget’s disease may be linked to obesity, radiation exposure, and are more common in Caucasians than individuals of other races
• The presence of a lesion (red skin, thickened) in the affected skin region is the most typical symptom associated with the condition. Individuals often report a burning and itchy sensation. Ulceration of the lesion can also be observed
• The treatment of choice for Secondary Cutaneous Extramammary Paget’s Disease is skin-directed therapy and treating the underlying malignancy. Surgery, radiation therapy, and chemotherapy may be used to treat the underlying malignancy
• The prognosis for Secondary Cutaneous Extramammary Paget’s Disease depends on the stage of the underlying malignancy. In individuals who have advanced stage of underlying tumors, the prognosis is guarded
• This disease is named after Sir James Paget, who first described it. Several other diseases also bear his name, so readers must be careful to not confuse them. Sometimes the spelling used is “Paget’s” disease, sometimes it is called “Paget disease”

Who gets Secondary Cutaneous Extramammary Paget’s Disease? (Age and Sex Distribution)

• Secondary Cutaneous Extramammary Paget Disease is rare and is typically observed in older adults
• Both males and females are affected
• Individuals of all races and ethnic groups around the world are affected. Although, Caucasians are observed to have a higher incidence of the condition

What are the Risk Factors for Secondary Cutaneous Extramammary Paget’s Disease? (Predisposing Factors)

Specific risk factors associated with Secondary Cutaneous Extramammary Paget Disease are currently not known. However, the following factors may play a role in the development of Paget’s disease:

• Advanced age
• Exposure to radiation
• Obesity
• Belonging to the Caucasian race

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Secondary Cutaneous Extramammary Paget’s Disease? (Etiology)

The cause of Secondary Cutaneous Extramammary Paget Disease is due to an underlying malignancy.

• It is the expression of certain underlying malignancies (or cancers) manifesting as skin conditions; i.e., the skin involvement is seen secondary to primary malignancy
• Such malignancies may include carcinomas of the female genitalia (cervix, vagina, Bartholin glands), prostate gland, rectum, adnexal carcinoma of skin, and transitional cell carcinomas of the urinary bladder and urethra

What are the Signs and Symptoms of Secondary Cutaneous Extramammary Paget’s Disease?

The signs and symptoms of Secondary Cutaneous Extramammary Paget’s Disease may include:

• Initially, it may appear like a superficial skin condition
• Red, inflamed skin rashes that itch and burn
• The skin patches or plaques are well-defined
• Eczema-like appearance of the skin lesion (scaly lesions)
• Ulceration of the lesion; bleeding
• Pain
• Commonly, the skin in the abdomen and pelvis including the perineum are involved, due to Paget’s disease originating from the colorectal or urothelial region (near the urinary tract)
• Signs and symptoms of the underlying malignancy may be noted

How is Secondary Cutaneous Extramammary Paget’s Disease Diagnosed?

A diagnosis of Secondary Cutaneous Extramammary Paget’s Disease may include:

• A complete physical examination and assessment of the individual’s medical history
• Ultrasound scan of the affected region
• Computed tomography (CT) and magnetic resonance imaging (MRI) scans, as necessary
• Tissue biopsy or skin biopsy: A biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
• Diagnostic tests to detect and diagnose underlying malignancies causing Secondary Cutaneous EMPD

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Secondary Cutaneous Extramammary Paget’s Disease?

The following may be some complications of Secondary Cutaneous Extramammary Paget’s Disease:

• Bleeding and ulceration might lead to bacterial and fungal infections
• Pain/discomfort during intercourse
• Stress, anxiety due to cosmetic concerns and cancer
• Uncommonly, tumor metastasis to the lymph nodes of the groin and other regions of the body may be noted
• Recurrence of the condition even after treatment - Paget’s disease is reported to recur and can become chronic
• Side-effects of chemotherapy and radiation therapy
• Complications due to underlying malignancy

How is Secondary Cutaneous Extramammary Paget’s Disease Treated?

The treatment measures involve those directed towards the skin and underlying malignancy. Skin-directed therapies for Secondary Cutaneous Extramammary Paget’s Disease are used (often combination therapies are used) that may include:

• Sunlight therapy (or exposure to ultraviolet light)
• Use of topical steroids and chemotherapy
• Systemic chemotherapy
• Radiation therapy to affected (local) skin area
• Biological therapy

Treatment of underlying malignancy causing Secondary Cutaneous EMPD may be undertaken through the following measures:

• In majority of cases, a wide surgical excision and removal of the entire tumor/lesion is the preferred treatment option. The surgical removal of the tumor depends on the size and stage of the condition
• Mohs micrographic surgery has been used for extramammary Paget’s disease. A review of a small number of patients indicated possible lower recurrence rates. The study was not large enough to confirm Mohs surgery as a preferred method
• This may be followed by radiation therapy and/or chemotherapy
• For metastasized tumors (in rare cases), a combination of chemotherapy, radiation therapy, and invasive procedures may be used
• In some cases, non-surgical treatment methods may be employed, which include radiotherapy, chemotherapy, laser therapy (using a CO₂ laser), and photodynamic therapy
• Counseling may be provided to help with stress and anxiety
• Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals

Follow-up care with regular screening and check-ups are important, since the tumor is known to recur in many cases.

How can Secondary Cutaneous Extramammary Paget’s Disease be Prevented?

Secondary Cutaneous Extramammary Paget’s Disease develops secondary to systemic disease; meaning that the EMPD affects other body organs, before affecting skin. No preventive methods have been reported for this condition. Nevertheless, maintaining a healthy lifestyle can help one avoid/delay the onset of the condition.

The following tips may be helpful:

• Maintain healthy lifestyle habits
• Lead an active life
• Eat a healthy diet
• Limit alcohol consumption and avoid smoking

What is the Prognosis of Secondary Cutaneous Extramammary Paget’s Disease? (Outcomes/Resolutions)

The prognosis of Secondary Cutaneous Extramammary Paget’s Disease depends on the severity and stage of the underlying malignancy.

• In general, the prognosis of the underlying disease/malignancy may depend on the following set of factors:
  • Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is poor
  • The size of the tumor: Individuals with small-sized tumors fare better than those with large-sized tumors
  • Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
  • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
  • Individuals with bulky disease have a poorer prognosis
  • Involvement of the lymph node can adversely affect the prognosis
  • Involvement of vital organs may complicate the condition
  • The surgical resectability of the tumor (meaning if the tumor can be removed completely)
  • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
  • Response to treatment of salivary gland cancer: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
  • Progression of the condition makes the outcome worse

Additional and Relevant Useful Information for Secondary Cutaneous Extramammary Paget’s Disease:

Paget’s disease arising in the skin outside the breast region is called primary cutaneous extramammary Paget’s disease. The condition involves the skin with associated sweat glands (frequently apocrine sweat glands) bearing skin and mostly occurs in the pelvic region (in and around the genitalia). Another completely different disease bearing Dr. Paget’s name s Paget’s disease of bone.