Summary about Disease
Extramammary Paget's Disease (EMPD) is a rare type of skin cancer that typically presents as a slow-growing, persistent rash, often around the vulva, scrotum, perineum, or perianal area. While it can be confined to the epidermis (outer layer of skin), it can sometimes indicate an underlying internal malignancy or spread deeper into the skin. EMPD is not usually life-threatening if treated early, but recurrence is possible.
Symptoms
The primary symptom of EMPD is a chronic, itchy, and often painful rash. Other symptoms can include:
Red, scaly, or eczematous patches
Crusting
Ulceration
Burning sensation
Nipple-like bumps
Bleeding
Causes
The exact cause of EMPD is not always clear. It can arise in a few ways:
Primary EMPD: Arising spontaneously in the skin of the affected area.
Secondary EMPD: Occurring due to the spread of cancer cells from an internal organ (like the bladder, rectum, or other genitourinary cancers) to the skin.
Intraepidermal Extension: From nearby cancer like anal or urethral cancer.
Medicine Used
Treatment options vary based on the extent and location of the disease, and if it is primary or secondary. Common treatments include:
Topical Medications: Imiquimod cream (an immune response modifier) or chemotherapy creams.
Surgical Excision: Wide local excision to remove the affected skin.
Radiation Therapy: Used if surgery is not possible or to treat residual disease.
Photodynamic Therapy (PDT): Uses a light-sensitizing drug and a special light to destroy cancer cells.
Chemotherapy: If there is spread to internal organs.
Mohs Surgery: A precise surgical technique that removes skin layer by layer until only cancer-free tissue remains.
Is Communicable
No, Extramammary Paget's Disease is not communicable or contagious. It cannot be spread from person to person.
Precautions
There are no specific precautions to prevent EMPD, as the cause is not fully understood. However, early detection and treatment are crucial. Regular skin self-exams and prompt consultation with a dermatologist for any persistent, unusual skin changes are recommended.
How long does an outbreak last?
EMPD is not an "outbreak" like an infection. It is a chronic condition that will persist and potentially worsen if left untreated. Without treatment, the affected area will continue to exhibit symptoms. The duration of symptoms varies greatly from person to person and is dependent on the stage and treatment intervention.
How is it diagnosed?
Diagnosis typically involves:
Physical Exam: Examination of the skin.
Skin Biopsy: A small sample of the affected skin is removed and examined under a microscope to confirm the presence of Paget cells.
Immunohistochemistry: Special stains are used on the biopsy sample to help differentiate EMPD from other skin conditions.
Imaging Studies: If secondary EMPD is suspected, imaging tests (CT scans, MRI, PET scans, colonoscopy, cystoscopy) may be ordered to look for an underlying internal cancer.
Timeline of Symptoms
EMPD progresses slowly, often over months or years. The timeline can vary:
Initial Stage: Begins as a small, itchy, red or scaly patch.
Progression: The patch gradually enlarges, becomes more inflamed, and may develop crusts, ulcerations, or bleeding. The area can become increasingly painful and uncomfortable.
Advanced Stage: If left untreated, the disease can spread deeper into the skin and potentially involve lymph nodes or internal organs (in secondary EMPD).
Important Considerations
Rule out underlying malignancy: A thorough evaluation is crucial to determine if the EMPD is primary or secondary to an internal cancer.
Long-term follow-up: Recurrence is possible, so regular follow-up appointments with a dermatologist or oncologist are essential after treatment.
Psychological Impact: The chronic nature of the disease and its location can have a significant impact on a person's quality of life and self-esteem. Addressing these issues through counseling or support groups may be helpful.
Individualized Treatment: There is no one-size-fits-all treatment. The best course of action depends on the specific characteristics of the case.