What to Know About Pyoderma Gangrenosum

This painful skin condition is often associated with autoimmune diseases

Pyoderma gangrenosum (PG) is a rare, chronic skin condition that causes a painful rash leading to ulcers or open sores. Initially, the rash may look like a blood pimple or a blood blister that won’t go away. Over time, skin around the blister deteriorates, leaving an open wound.

Pyoderma gangrenosum is most common in people with other autoimmune or inflammatory conditions, including inflammatory bowel syndrome or arthritis. The cause is unknown and difficult to diagnose because it resembles many other conditions. Medications and other treatments can relieve symptoms.

Continue reading to learn more about pyoderma gangrenosum treatment and to see pictures that might help you determine whether you could have pyoderma gangrenosum. 

Male doctor wrapping bandage on older woman's need

Pichsakul Promrungsee / Getty Images

How Does Pyoderma Gangrenosum Affect the Body?

Pyoderma gangrenosum leads to painful open wounds around the body. At first, patients notice a small rash or blood blister. Sometimes, these occur after minor scratches or skin injuries. The affected area quickly expands, filling with dead cell matter called fibrinous debris.

The ulcers often have a blue or purple border. They usually appear on the legs but can be anywhere, including the groin and genitals.

Healthcare providers believe that pyoderma gangrenosum is caused by an overactive immune system, and researchers suspect it’s an autoimmune disease.

Pyoderma Gangrenosum

Types of Pyoderma Gangrenosum

Most people with pyoderma gangrenosum have what's known as classical, or classic, pyoderma gangrenosum. It usually occurs on the legs and trunk. It can lead to deep ulcers that are very painful. This type makes up about 85% of pyoderma gangrenosum cases.

Within classic pyoderma gangrenosum, there’s another subtype: peristomal pyoderma gangrenosum. This occurs when pyoderma gangrenosum develops around surgical openings. 

The three other types of pyoderma gangrenosum are:

  • Atypical or bullous pyoderma gangrenosum: This type mostly affects the hands, and presents with blisters. It happens most often in people who have blood cancers like leukemia. 
  • Pustular pyoderma gangrenosum: This type occurs most often on the arms of legs. It begins with pustules that become ulcers, and is easy to confuse with classic pyoderma gangrenosum. Pustular pyoderma gangrenosum is common in people who have inflammatory bowel disease (IBD).  
  • Vegetative pyoderma gangrenosum: This type, also known as superficial pyoderma gangrenosum, causes ulcers that are not particularly deep or painful. It usually develops in people with no underlying health concerns.

Symptoms of Pyoderma Gangrenosum

The most prominent symptom of pyoderma gangrenosum is open ulcers filled with fibrinous debris. People with atypical pyoderma gangrenosum might not develop these ulcers. These ulcers usually start with a small rash that spreads quickly. The ulcers might continue spreading or heal on their own.

In addition to ulcers, other symptoms of pyoderma gangrenosum include:

  • Fever
  • Localized pain around the rash and ulcers
  • Feeling generally unwell
  • Joint pain

Who Does Pyoderma Gangrenosum Affect?

Pyoderma gangrenosum is most common in middle-aged women. Half of the people diagnosed with pyoderma gangrenosum have another systemic health condition. Overall, it impacts about 1 in 100,000 Americans each year.

What Conditions Is Pyoderma Gangrenosum Associated With?

Pyoderma gangrenosum is closely associated with:

Is Pyoderma Gangrenosum Life-Threatening?

Pyoderma gangrenosum is a serious disease that can be life-threatening, especially if left untreated. Compared to the overall population, people with pyoderma gangrenosum are three times more likely to die than their peers of the same age.

What Causes Pyoderma Gangrenosum?

Healthcare providers are still working to identify what causes pyoderma gangrenosum. However, many researchers believe that it's an autoimmune disease and its symptoms are caused by a malfunctioning immune system.

Specifically, pyoderma gangrenosum might be caused by an issue with neutrophils, the most common type of white blood cell. These cells respond to viruses, bacteria, and other pathogens to protect the body from infection.

How Is Pyoderma Gangrenosum Diagnosed?

There aren’t any specific tests for pyoderma gangrenosum. This can make it difficult to diagnose. Healthcare providers usually do a physical examination and eliminate other possible conditions before settling on a diagnosis of pyoderma gangrenosum. 

Possible Medications and Treatments for Pyoderma Gangrenosum

You should see a healthcare provider if you have signs of pyoderma gangrenosum, which includes a blood blister that won’t go away or an ulcer that isn’t healing. Pyoderma gangrenosum needs medical management, so when in doubt, see your primary care physician or a dermatologist. 

There’s no specific treatment protocol for pyoderma gangrenosum. Your healthcare provider will consider your individual case and any underlying health conditions you have to find a treatment that will work for you. Pyoderma gangrenosum is a chronic disease, and you may experience more ulcers in the future.

Dermatologists often use these treatments to manage pyoderma gangrenosum:

  • Corticosteroids, which reduce inflammation and can be applied topically to the ulcer and affected skin area
  • Tacrolimus, an eczema drug can reduce inflammation and immune response
  • Dapsone, an antibiotic, anti-inflammatory drug
  • Cyclosporine, an immunosuppressant drug that’s often used in transplant patients

Pyoderma Gangrenosum Self-Care

Living with pyoderma gangrenosum can be quite painful. These steps can help you manage the physical pain and day-to-day impact of pyoderma gangrenosum:

Pyoderma Gangrenosum Outlook

Pyoderma gangrenosum is a chronic condition, so once you have it, you’ll likely need to manage it throughout your life. It can be an unpredictable condition to live with and has some scary implications—including a death rate that’s higher than it is for people without the condition.

However, with management and a trusted healthcare provider, you can live a relatively typical life span with pyoderma gangrenosum.

When to Contact a Healthcare Provider

If you believe you have pyoderma gangrenosum, see a healthcare provider right away. You can visit your primary care provider, but they may refer you to a specialist, such as a dermatologist. These doctors are knowledgeable about the skin, as well as the autoimmune conditions that can impact skin health. 

Summary

Pyoderma gangrenosum (PG) is a chronic skin condition that can cause painful ulcers. These ulcers usually occur on the legs and trunk but can develop anywhere. About half of pyoderma gangrenosum cases develop in people with other autoimmune diseases, including inflammatory bowel disease and arthritis. While the medical community believes that pyoderma gangrenosum is linked to immune dysfunction, just around half of patients with pyoderma gangrenosum don’t have any other systemic diseases.

There are no specific tests for pyoderma gangrenosum, so healthcare providers will likely try to rule out other skin conditions before diagnosing pyoderma gangrenosum. Treatments include steroids, eczema drugs, and immunosuppressants. Pain management and wound care are also important. Although pyoderma gangrenosum can increase your risk of death, getting treatment and seeing your care team regularly can make it possible to live a full, healthy life. 

7 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  1. National Organization for Rare Diseases. Pyoderma gangrenosum.

  2. George C, Deroide F, Rustin M. Pyoderma gangrenosum – a guide to diagnosis and management. Royal College of Physicians Clinical Medical Journal. May 2019.

  3. Syed, Zeib, Shahrukh Syed, and Katherine Burmaster. “A case report of localized vegetative pyoderma gangrenosum of the foot in a patient with myasthenia gravis in an outpatient setting.Cureus. doi: 10.7759/cureus.41181.

  4. American Osteopathic College of Dermatology. Pyoderma gangrenosum.

  5. Gameiro, Ana, Neide Pereira, José Carlos Cardoso, and Margarida Gonçalo. “Pyoderma gangrenosum: challenges and solutions.Clinical, Cosmetic and Investigational Dermatology 8. 2015. Doi: 10.2147/CCID.S61202.

  6. Croitoru, David, Sheida Naderi-Azad, Muskaan Sachdeva, Vincent Piguet, and Afsaneh Alavi. “A wound care specialist’s approach to pyoderma gangrenosum.” Advances in Wound Care. 2020. doi.org: 10.1089/wound.2020.1168.

  7. DermNetNZ. Pyoderma gangrenosum.

By Kelly Burch
Kelly Burch is has written about health topics for more than a decade. Her writing has appeared in The Washington Post, The Chicago Tribune, and more.