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Definition
Pyoderma gangrenosum is a condition that causes large, painful sores (ulcers) to develop on your skin, most often on your legs.
It's not certain what causes pyoderma gangrenosum, but it may be related to a disorder of the immune system. About half the people with pyoderma gangrenosum have an underlying chronic health condition.
Pyoderma gangrenosum is rare. Effective treatment for pyoderma gangrenosum is available, but in some cases, the ulcers may leave scars. Early diagnosis and therapy reduce your risk of scarring. In some cases, pyoderma gangrenosum may recur.
Symptoms
Signs and symptoms of pyoderma gangrenosum include:
- Initially, one or more small, red bumps on your skin that resemble spider bites
- Painful, open sores (ulcers) with reddish-purple borders
- Achy joints or pain in your bones
- General feeling of unwellness (malaise)
The ulcers usually appear on your legs, but may develop anywhere on your body. 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 unexplained blistering on your skin, an itchy rash or a sore that's slow to heal.
Causes
The cause of pyoderma gangrenosum isn't well understood. It's often associated with inflammatory conditions, such as ulcerative colitis and Crohn's disease. If you have pyoderma gangrenosum, new skin trauma, such as a cut or puncture wound, may result in the formation of new ulcers. This tendency of new ulcers to form from trauma, including surgery, is known as pathergy.
Risk factors
Pyoderma gangrenosum is most common in people in their 40s and 50s, but can occur at any age. It's also associated with inflammatory conditions, including:
- Ulcerative colitis. This disease of the large intestine causes chronic inflammation of your large intestine.
- Crohn's disease. Like ulcerative colitis, Crohn's disease causes long-term inflammation that can occur anywhere along your digestive tract.
- Rheumatoid arthritis. Rheumatoid arthritis refers to a condition in which the thin membranes surrounding your joints become irritated and inflamed.
- Hepatitis. This condition causes your liver to become inflamed. The cause of hepatitis may be a virus or a disorder of your immune system.
Complications
Ulcers caused by pyoderma gangrenosum are usually raw and tender. Skin scarring is common.
Preparing for your appointment
You're likely to start by seeing your family doctor or a general practitioner. However, in some cases when you call to set up an appointment, you may be referred immediately to a doctor who specializes in treating skin conditions (dermatologist).
Here's some information to help you prepare for your appointment.
What you can do
- Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
- Write down key personal information, including any major stresses or recent life changes.
- Make a list of all medications, vitamins or supplements you're taking.
- Write down questions to ask your doctor.
Preparing a list of questions for your doctor will help you make the most of your time together. List your questions from most important to least important. For pyoderma gangrenosum, some basic questions to ask your doctor include:
- What is likely causing my symptoms or condition?
- Other than the most likely cause, what are other possible causes for my symptoms or condition?
- What kinds of tests do I need?
- Is my condition likely temporary or chronic?
- What is the best course of action?
- What are the alternatives to the primary approach you're suggesting?
- I have these other health conditions. How can I best manage them together?
- Are there any restrictions that I need to follow?
- Is there a generic alternative to the medicine you're prescribing?
- Are there any brochures or other printed material that I can take home with me? What websites do you recommend?
Don't hesitate to ask other questions during your appointment.
What to expect from your doctor
Your doctor is likely to ask you a number of questions, including:
- When did you begin experiencing symptoms?
- Have your symptoms been continuous or occasional?
- What, if anything, seems to improve your symptoms?
- Have you used any prescription medication for this condition? If so, what?
- What, if anything, appears to worsen your symptoms?
- Did you have any damage to your skin, such as a cut, bruise or burn, in the area of new a lesion?
- Do you have any inflammatory medical conditions, such as Crohn's disease, ulcerative colitis or rheumatoid arthritis?
What you can do in the meantime
Protect your skin from trauma. Avoid activities in which you put your skin at risk. Treat ulcers with antibacterial cream.
Tests and diagnosis
The sudden appearance of unexplainable ulcers on your skin is the first indication that you may have pyoderma gangrenosum. Tests may include:
- A physical exam.
- Blood tests and other tests to determine whether you have an underlying health condition that's associated with pyoderma gangrenosum.
- A skin biopsy, which may help support the diagnosis or rule out another cause. In this test, your doctor removes a small sample of the affected skin. The skin sample is examined under a microscope. The finding themselves often aren't specific, but they may support a diagnosis of pyoderma gangrenosum.
Treatments and drugs
If you have an underlying disease associated with pyoderma gangrenosum, treating that condition may help control the ulcers. However, other treatments may be necessary to heal the wounds.
Drugs
Your doctor may prescribe the following:
- Corticosteroids. Corticosteroids help relieve inflammation. Usually pyoderma gangrenosum is treated with oral corticosteroids, such as prednisone. Side effects of corticosteroids may include increased blood pressure, weight gain with fat deposits in your abdomen, face and back of your neck, and increased risk of infection. In some milder cases, a topical steroid — a medication you apply to your skin — may be used. Side effects may include skin thinning and acne.
- Immunosuppressant drugs. These drugs help calm the autoimmune response in your skin, which reduces inflammation. Because these drugs suppress your immune system, they can place you at higher risk of infections.
- Nonsteroidal anti-inflammatory medications. Medications in the sulfone family, such as dapsone, are often used. Milder cases might involve using minocycline, an anti-inflammatory and antibiotic medication.
- Tumor necrosis factor inhibitors. Drugs such as infliximab (Remicade), etanercept (Enbrel) and adalimumab (Humira) have shown some success in treating pyoderma gangrenosum, especially in people who have associated inflammatory bowel disease. Remicade is given by infusion, and Enbrel and Humira are given as injections. These drugs also can make you more vulnerable to infection and to certain cancers.
- High-dose intravenous immunoglobulin. When corticosteroids aren't enough to control pyoderma gangrenosum, intravenous immunoglubulin has shown promise as a treatment.
As your skin heals, you'll likely taper off the corticosteroids or immunosuppressants. You can expect your skin to recover several months after beginning therapy. Without treatment, the ulcers may widen, remain the same or slowly heal.
Surgery
Doctors don't commonly use surgery as a treatment option because trauma to the skin may worsen existing ulcers or stimulate new ones to develop. This tendency to worsen after surgery is called pathergy. Doctors may perform surgery to carefully remove dead or necrotic tissue, and once the inflammation has improved consideration could be given to skin grafting — if the ulcers on your skin are large and need help with healing, your doctor may surgically attach a piece of real or artificial skin over the open sores — but most often the wounds heal without grafting.
Lifestyle and home remedies
If you have pyoderma gangrenosum, it's important to treat your skin gently. These steps can help.
- Apply bandages and antibacterial creams as directed by your doctor. Careful treatment helps reduce the risk of scarring.
- Avoid injury to your skin. Wear protective clothing if you plan to do something that may put your skin at risk.
Coping and support
With treatment, you're likely to recover from pyoderma gangrenosum. However, you may feel stress about the possibility of recurrence or about the scarring of your skin. Here are some suggestions for coping with the disease:
- Educate yourself about pyoderma gangrenosum. The more you know, the better prepared you'll be to deal with complications or recurrences. Besides talking to your doctor, you may want to talk to a counselor or medical social worker. Or you may find it helpful to talk to other people with pyoderma gangrenosum.
- Follow your doctor's recommendations. If your doctor recommends certain treatments or lifestyle changes, be sure to follow them. Ask questions if anything is unclear.
- Maintain a strong support system. Family and friends can help you tremendously as you go through this difficult time. Sometimes, though, you may find the concern and understanding of other people with pyoderma gangrenosum especially comforting. Go online to connect with other people living with the disease.
Prevention
You can't prevent pyoderma gangrenosum. If you have the condition, try to avoid injuring your skin. Injury or trauma to your skin can provoke new ulcers to form.
Last Updated: 2010-06-12
