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Pyoderma gangrenosum

Overview

Pyoderma gangrenosum (pie-o-DUR-muh gang-ruh-NO-sum) is a rare condition that causes large, painful sores (ulcers) to develop on your skin, most often on your legs.

No one knows exactly what causes pyoderma gangrenosum, but it appears to be a disorder of the immune system. People who have certain underlying conditions, such as inflammatory bowel disease or rheumatoid arthritis, are at higher risk of pyoderma gangrenosum.

The ulcers of pyoderma gangrenosum can develop quickly. They usually clear up with treatment, but scarring and recurrences are common.

Advanced pyoderma gangrenosum

Pyoderma gangrenosum can cause painful, open sores (ulcers) that have bluish, overhanging borders.

Symptoms

Pyoderma gangrenosum usually starts with a small, red bump on your skin, which may resemble a spider bite. Within days, this bump can develop into a large, painful open sore.

The ulcer usually appears on your legs, but may develop anywhere on your body. Sometimes it appears around surgical sites. If you have several ulcers, they may grow and merge into one larger ulcer.

When to see a doctor

Talk to your doctor if you develop a painful, rapidly growing skin wound.

Causes

Pyoderma gangrenosum isn't infectious or contagious, but no one knows exactly what causes it. It's often associated with autoimmune diseases such as ulcerative colitis, Crohn's disease and rheumatoid arthritis. And some studies indicate that it may have a genetic component.

If you have pyoderma gangrenosum, new skin trauma, such as a cut or puncture wound, may trigger new ulcers.

Risk factors

Certain factors may increase your risk of pyoderma gangrenosum, including:

  • Your age and sex. The condition is slightly more common among women. And it usually occurs between the ages of 40 and 50, though it can appear at any age.
  • Having inflammatory bowel disease. People with a digestive tract disease such as ulcerative colitis or Crohn's disease are at increased risk of pyoderma gangrenosum.
  • Having rheumatoid arthritis. People with rheumatoid arthritis are at increased risk of pyoderma gangrenosum.
  • Having blood cancer. People with blood disorders (hematologic malignancies) are at increased risk of pyoderma gangrenosum.

Complications

Possible complications of pyoderma gangrenosum include:

  • Infection
  • Scarring
  • Uncontrolled pain
  • Depression
  • Loss of mobility

Diagnosis

Your doctor will talk with you about your medical history and conduct a physical exam. No test can confirm a diagnosis of pyoderma gangrenosum. But your doctor may order a variety of tests to rule out other conditions that may have similar signs or symptoms.

  • Blood tests. A sample of your blood can be tested for evidence of infection, liver or kidney problems, and rheumatoid arthritis, among other conditions.
  • Skin biopsy. A skin biopsy involves removing a small sample of the affected skin so that it can be viewed under a microscope.
  • Colonoscopy. An examination of your colon can check for inflammatory bowel disease.
  • Imaging studies. A CT scan or chest X-ray may be used to check for deep infection or inflammation.

Treatment

Treatment of pyoderma gangrenosum is aimed at reducing inflammation, controlling pain and promoting wound healing. Depending on the size and depth of your skin ulcers, it can take weeks or months for them to heal, often with scarring. Treatment may also involve a hospital stay or specialized wound care in a burn treatment center.

Even after successful treatment, it's common for new wounds to develop.

Medications

  • Corticosteroids. High doses of corticosteroids are the mainstay of pyoderma gangrenosum treatment. These drugs may be applied to the skin, injected into the wound or taken by mouth (prednisone). Using corticosteroids for a long time or in high doses may cause serious side effects, including bone loss and an increased risk of infection. To help limit side effects, your doctor will gradually reduce your dose once your wounds begin to heal.
  • Drugs that suppress the immune system. Another way to limit your prednisone dose is to also use drugs that suppress the immune system, such as calcineurin inhibitors (tacrolimus), cyclosporine, mycophenolate and infliximab. Depending on the type of drug used, it may be applied to the wounds, injected or taken by mouth.
  • Pain medication. Depending on the extent of your wounds, you may benefit from pain medication, especially when dressings are being changed.

Wound care

In addition to applying medicine directly to your wounds, your doctor or wound care specialist will cover them with a moist (not wet or dry) dressing and, perhaps, an elasticized wrap. You may be asked to keep the affected area elevated.

Follow your doctor's instructions carefully regarding wound care. This is especially important because many of the oral medications prescribed for pyoderma gangrenosum suppress your immune system, which increases your risk of infection.

Surgery

Because pyoderma gangrenosum can be made worse by cuts to the skin, surgery to remove dead tissue is not usually considered a good treatment option. Trauma to the skin may worsen existing ulcers or trigger new ones.

If the ulcers on your skin are large and need help with healing, your doctor might suggest a skin graft. In this procedure, the surgeon attaches a piece of skin or synthetic skin over the open sores. This is attempted only after the wound inflammation has gone and the ulcer has started healing.

Preparing for an appointment

You may start by seeing your primary care physician. Or you may be referred immediately to a doctor who specializes in skin conditions (dermatologist).

What you can do

Before your appointment, you may want to write down answers to questions your doctor is likely to ask, such as:

  • When did your symptoms begin?
  • Have you had similar symptoms in the past? If so, what treatment worked then?
  • Have you tried any treatments on your wound? Have they helped?
  • What medications and supplements do you take regularly?
  • Did you have any damage to your skin, such as a puncture or cut, in the area where the sore developed?
  • What other medical conditions do you have?

Coping and support

With treatment you're likely to recover from pyoderma gangrenosum. You may feel depressed if the process takes a long time and is painful. Or you may feel stressed about the possibility of recurrence or about how your skin looks. You may find it helpful to talk with a counselor, medical social worker or other people who have or had pyoderma gangrenosum.

If you want counseling or support, ask your doctor for a referral to a mental health professional or contact information for a support group in your area.

Prevention

You can't totally prevent pyoderma gangrenosum. If you have the condition, try to avoid injuring your skin. Injury or trauma to your skin, including from surgery, can provoke new ulcers to form. It may also help to control any underlying condition that may be causing the ulcers.

Last updated: December 16th, 2015

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