Extramammary Paget's disease

Overview

Extramamary Paget’s disease is first reported by Dr. Radcliffe Crocker in 1889. This disease is eczematoid changes in the skin of vulva, penis, greater lip of pudendum and crissum, etc. Usual symptoms include redness, tingling, itching, increased sensitivity, burning, and pain of the skin. Treatments involve surgery, radiation therapy, or a combination of them.

What are the symptoms of Extramammary Paget’s disease?

Usual signs and symptoms are:

  • Eczematoid changes in the area skin
  • Tingling, itching, increased sensitivity, burning, and pain of the skin

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

Who is at highest risk?

The cause of Extramammary Paget’s disease (EMPD) is unknown.

When to seek urgent medical care?

Call your health care provider if symptoms of extramammary Paget’s disease develop. If you experience either of the following symptoms, seeking urgent medical care as soon as possible:

  • Skin irritation,
  • Redness, or
  • Scaliness of the skin

Diagnosis

  • Biopsy: Biopsy is the most important test for the diagnosis. During this peroid, the doctors remove a small amount of tissue for examination under a microscope. Then the pathologists may analyze the samples and tell whether the tissues is benign or cancerous, what kind of cancer it is.
  • Computed tomography (CT) scan and biopsy: CT scans are often used to diagnose extramamary Paget’s disease. It can confirm the location of the cancer and show the organs nearby, as well as lymph nodes and distant organs where the cancer might have spread. These are helpful for determining the stage of the cancer and in determining whether surgery is a good treatment option. CT scans can also be used to guide biopsy and a biopsy sample is usually removed and looked at under a microscope.
  • Magnetic resonance imaging (MRI): An MRI uses magnetic fields but it is a different type of image than what is produced by computed tomography (CT) and produces detailed images of the body. Like computed tomography (CT), a contrast agent may be injected into a patient’s vein to create a better picture.
  • Chest X-ray: This plain x-ray of your chest may be done to see if the cancer has spread to your lungs.
  • Whole Bone Scan: The goal of a whole body bone scan is to show if a cancer has metastasized to your bones.

Treatment options

Patients with extramammary Paget’s disease have many treatment options. The selection depends on the stage of the tumor. The options are surgery, radiation therapy, or a combination of these methods. Before treatment starts, ask your health care team about possible side effects and how treatment may change your normal activities. Because cancer treatments often damage healthy cells and tissues, side effects are common. Side effects may not be the same for each person, and they may change from one treatment session to the next.

  • Surgery: This is the main treatment for the disease. The goal is to remove the tumor and some surrounding tissue to make sure that the entire tumor is removed. The indication of surgery depends on the stage of the cancer and the patient’s general health.
  • Radiation therapy: This is a cancer treatment to kill cancer cells or keep them from growing by using high-energy x-rays or other types of radiation.

Diseases with similar symptoms

  • Basal Cell Carcinoma
  • Intertrigo
  • Lichen simplex chronicus
  • Candidiasis
  • Contact Dermatitis
  • Tinea Cruris

Where to find medical care for Extramammary Paget’s disease?

Ask our experts on Extramammary Paget’s disease

What to expect (Outlook/Prognosis)?

The prognosis of extramamary Paget’s disease depends on the following:

  • Whether or not the tumor can be removed by surgery
  • The stage of the cancer: the size of the tumor, whether the cancer has spread outside the vulva or penis
  • The patient’s general health
  • Whether the cancer has just been diagnosed or has recurred

Prevention

For the risk factors are not clear, the preventive measure is unknown.

Source

http://dermnetnz.org/site-age-specific/extra-mammary-paget.html

Attribution

This article incorporates public domain material from Wikidoc and MedlinePlus. Please see licenses for further details.

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