Pyoderma Gangrenosum
What is Pyoderma Gangrenosum?
Pyoderma gangrenosum (PG) is a rare non-infectious, autoinflammatory condition characterized by painful, rapidly expanding skin ulcers with violet-colored irregular edges. It typically occurs in association with systemic inflammatory or autoimmune diseases and a phenomenon known as pathergy, where a minor injury can become a larger ulcer. Despite its name, PG is neither caused by pyogenic (pus-producing) bacteria nor gangrenous (blood flow blockage to an area, causing tissue death).
Synonyms
- Ulcerative neutrophilic dermatosis
- Sterile neutrophilic ulceration
- Dermatitis gangrenosum
- Geometric phagedenism (historical)
Pyoderma gangrenosum (PG) is a rare non-infectious, autoinflammatory condition characterized by painful, rapidly expanding skin ulcers with violet-colored irregular edges. It typically occurs in association with systemic inflammatory or autoimmune diseases and a phenomenon known as pathergy, where a minor injury can become a larger ulcer. Despite its name, PG is neither caused by pyogenic (pus-producing) bacteria nor gangrenous (blood flow blockage to an area, causing tissue death).
PG has an estimated global prevalence of 1 case per 100,000 individuals. It most commonly affects adults aged 30-60 years with a slight female predominance. Pediatric cases account for about 4% of patients.
| Name | Abbreviation |
|---|---|
| Ulcerative neutrophilic dermatosis | |
| Sterile neutrophilic ulceration | |
| Dermatitis gangrenosum | |
| Geometric phagedenism (historical) |
An exact cause for PG is unknown. The disease involves a complex interplay between genetic predisposition and a dysregulated immune response. Key features include over-activation of neutrophils (a type of white blood cells), elevated levels of cytokines (immune cell signaling molecules) such as TNF-α, IL-1β, IL-8, IL-17, IL-23, IL-36, and impaired wound healing. Associated conditions such as inflammatory bowel disease, rheumatoid arthritis, blood cancers or other autoimmune diseases are present in about 50-70% of cases. Triggers may include pathergy (trauma to skin resulting in new lesions), surgery or medications. Variants in inflammatory regulator genes such as PSTPIP1, NLRP3, NLRP12, NOD2 and JAK2 may play a role in a predisposition to PG.
Clinical presentation of PG may include the following:
Initial Stage:
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Small pustule, papule, or nodule that rapidly breaks down
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Sometimes begins as tender, erythematous area which can expand into a larger lesion over several days
Established Lesions:
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Rapidly enlarging painful ulcers with irregular, violaceous (purple) borders
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Necrotic (dead cell) base with purulent or hemorrhagic exudate
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Cribriform (sieve-like) scarring as lesions heal
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Surrounding erythema and edema
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Healing can be slow, about half of patients heal by six months, others take longer
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About one-third of patients may experience a recurrence by 1.6 years
Distribution:
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Lower extremities (legs) most common - approximately 70% of cases
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Can occur anywhere including trunk, arms, face, and genitals
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May be single or multiple lesions
Clinical Variants:
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Ulcerative (classic) - most common, as described above
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Bullous - associated with hematologic malignancies, fluid-filled vesicles
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Pustular - multiple small pustules, associated with inflammatory bowel disease
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Vegetative (superficial) - less aggressive, single plaque with minimal ulceration
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Peristomal - occurs around stomas (surgical openings)
Associated Symptoms:
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Severe pain, often out of proportion to appearance
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Fever and malaise in severe cases
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Pathergy phenomenon
There is no definitive diagnostic test for PG. Diagnosis is by exclusion and ruling out of other causes. The diagnostic approach may include:
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A thorough clinical assessment and history
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Skin biopsy showing sterile neutrophilic infiltrate
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Exclusion of infection, vasculitis, malignancy, and other ulcerative conditions
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Laboratory evaluation for associated systemic disease
Diagnostic scoring frameworks such as the Delphi or PARACELSUS criteria are often used to support a diagnosis.
Treatment of PG is aimed at suppressing the underlying autoinflammatory response, controlling pain, facilitating wound healing and managing associated diseases. Measures include gentle wound care; pain medications; immunosuppressive drugs such corticosteroids, methotrexate, cyclosporine, and topical tacrolimus; and biologics such as TNF (tumor necrosis factor), C5a, IL-1, IL-6, IL-12/23, IL-36 and JAK inhibitors. Treatment is based on disease severity and often requires prolonged therapy. Surgical interventions are generally avoided due to pathergy risk.
The prognosis for PG patients is variable and relapses can occur in 25-30% of cases. Many respond well to immunosuppressive therapy. Lesions may take weeks to months to heal. Delayed diagnosis or inappropriate surgical treatment may worsen outcomes. Scarring is common after healing. Mortality is rare and usually related to associated systemic disease rather than PG itself.
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Maronese, C. A., Pimentel, M. A., Li, M. M., Genovese, G., Ortega-Loayza, A. G., & Marzano, A. V. (2022). Pyoderma Gangrenosum: An Updated Literature Review on Established and Emerging Pharmacological Treatments. American journal of clinical dermatology, 23(5), 615–634. https://doi.org/10.1007/s40257-022-00699-8.
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Wollina U. (2007). Pyoderma gangrenosum--a review. Orphanet journal of rare diseases, 2, 19. https://doi.org/10.1186/1750-1172-2-19.
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Maverakis E, Ma C, Shinkai K, Fiorentino D, Callen JP, Wollina U, Marzano AV, Wallach D, Kim K, Schadt C, Ormerod A, Fung MA, Steel A, Patel F, Qin R, Craig F, Williams HC, Powell F, Merleev A, Cheng MY. Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum: A Delphi Consensus of International Experts. JAMA Dermatol. 2018 Apr 1;154(4):461-466. doi: 10.1001/jamadermatol.2017.5980. PMID: 29450466.
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National organization for rare disorders (NORD): Pyoderma gangrenosum.
Last updated 18 Jul 2011, 09:03 PM
18 Jul 2011, 09:03 PM
https://www.facebook.com/groups/368163159232?ap=1 and https://www.facebook.com/groups/56007379235?ap=1 are available to PG patients on Facebook! Plenty of info and support are available there. Best, Harley
Last updated 4 Jun 2009, 12:44 PM
4 Jun 2009, 12:44 PM
Hi Kerry, I don't mind posting my email address on here, annscd08@yahoo.com, if you want to contact me. It may be more helpful for others though to discuss successful treatments here. I'm still struggling with healing the ankle ulcer so I'll look into the Hydrablue that you mentioned. I haven't been able to wear a full shoe for a year now. I would love to go the beach so I need to get this healed. What nutritional supplements did you use? I found that when I started the SCD diet that my old scar healed up completely and now you cannot even see where it was. I'm hoping the same thing for my ankle. The first one happened with a tiny nick from a razor when shaving my leg and the second happened when I had an accupuncture treatment. Maybe having some of that hydrablue on hand will make me feel more confident, just in case. I went to a homeopathic doctor today for the first time for immune system treatments. He says that the pyoderma is the immune system reacting at the cellulare level and seems to believe he can get my immune system in balance with various homeopathic treatments. I'll let you know how that goes. Please try the SCD diet for your daughter. It's a healthy diet and it works wonders for many inflammatory diseases, and even autisim. It's a bit strict but well worth it. There is a website for parents of kids on the SCD diet called pecanbread, it has a lot of great info on it.
4 Jun 2009, 10:10 AM
Thanks for all the information Shirley Ann. I can't figure out from this site how to contact you directly. As I mentioned in response to your other post, we had great success with nutritional supplements (some of which we ground up and applied topically) as my daughter did not respond well to Prednisolone. We had been told it might take up to a year to heal the ulcers but managed to do so in 10 weeks and had great success with the scar tissue too. Would be happy to tell you in detail what we did. But not sure how I can email you directly?
3 Jun 2009, 03:23 PM
I've had only two episodes of PG and pray God to never have another one. I have been without medical help as no one here has ever heard of it or treated it and a plastic surgeon in the hospital cut away some skin without my permission and against my protest that it would exascerbate it, which it did, turning 2 ulcers into 10 new ones. I found relief by searching internet research articles and I treated it successfully with prednisone, .5 milligram per kilo of my body weight. The ulcers responded immediately but still have taken a full year to heal and are not healed fully yet. They have closed but the skin is still very tender and I can't wear shoes as it's on my ankle. I also used an over the counter steroid/antibiotic cream. I have heard that it's often related to IBD, which I have, Crohn's, and each bout of PG came after a flare-up of the Crohn's. So I sought a cure for the Crohn's that was not a deadly immune suppressant and I found it in the the SCD, Specific Carbohydrate Diet. The book, Breaking the Viscious Cycle, has been a lifesaver for me. I have had no Crohn's symptoms for the past 10 months I've been on the diet. I now take no medicine for the disease, only diet. It's the first time in 30 years that I have been free of the Crohn's symptoms. In relieving the Crohn's I'm hoping that the PG will never appear again. Though I am always very fearful of a cut or puncture that may turn into this nightmare ulcer. If you want to know more about using diet to relieve auto immune diseases, you can contact me or search "SCD" or Specific Carbohydrate Diet, or Breaking the Vicious Cycle".
Last updated 4 Jun 2009, 10:05 AM
4 Jun 2009, 10:05 AM
Hi Shirley Ann, I'm interested to read about your success with diet. We have also found great success with nutritional supplements and detoxing our house so that all the personal care products we use have no harmful ingredients. My daughters scar is also healing really well which I put down to the Hydrofera blue dressing and a specific oil I use.
3 Jun 2009, 03:29 PM
I'm so sorry for your child who has this, I treat each scrape or scratch as if it may become PG, but I'm not really sure what to do except put on some steroid cream and bandaid and wait 5 days. I personally believe that if our sed rate is low and near the normal range scrapes won't turn into PG so I try very hard to keep my inflammation down with diet.
7 Apr 2009, 05:26 AM
As the mother of a pyoderma gangrenosum sufferer I find it very difficult to work out how to cope with scrapes and scratches. Do we need to treat each one as a potential lesion?
Last updated 7 Apr 2009, 05:27 AM
7 Apr 2009, 05:27 AM
Has anyone else used Hydrofera Blue wound dressing? We are trying to access it in Australia but having problems getting the hospital to order it?
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