Background

Pyoderma gangrenosum is an uncommon ulcerative cutaneous condition whose cause is uncertain. Although the etiology of this condition is poorly understood, dysregulation of the immune system (specifically, altered neutrophil chemotaxis) is believed to be involved. Pyoderma gangrenosum is associated with systemic diseases in at least 50% of patients who are affected.^{[1, 2, 3]}

The diagnosis is made by excluding other causes of similar-appearing cutaneous ulcerations, including infection, malignancy, vasculitis, vasculopathy, venous insufficiency, collagen-vascular diseases, diabetes, and trauma. In a process termed pathergy, new ulcerations may occur after trauma or injury to the skin in 30% of patients who already have pyoderma gangrenosum. (See Presentation, DDx, and Workup.)

Patients with pyoderma gangrenosum may have involvement of other organ systems that manifests as sterile neutrophilic infiltrates. Culture-negative pulmonary infiltrates are the most common extracutaneous manifestation.^{[4, 5]}Other organ systems that may be involved include the heart, the central nervous system (CNS), the gastrointestinal (GI) tract, the eyes, the liver, the spleen, the bones, and the lymph nodes. (See Presentation and Workup.)

Therapy for pyoderma gangrenosum involves the use of anti-inflammatory agents, including antibiotics, corticosteroids, immunosuppressive agents, and biologic agents. The prognosis is generally good; however, the disease can recur, and residual scarring is common. (See Prognosis, Treatment, and Medication.)

Next: Epidemiology

Epidemiology

Pyoderma gangrenosum occurs in about 1 in 100,000 persons each year in the United States. A Spanish study that examined hospital admissions for pyoderma gangrenosum as a percentage of total hospital admissions over the period 1999-2021 found that in 2021, pyoderma gangrenosum admissions accounted for 91.9 of every 1,000,000 admissions.^[6]

All ages may be affected by the disease, but it predominantly occurs in the fourth and fifth decades of life. Children account for only 3-4% of the total number of cases. Nothing is clinically distinctive about pyoderma gangrenosum in children and adolescents, other than the age of the patients. Although this condition is known to affect both sexes, a slight female predominance may exist.^{[7, 8, 6]}

Next: Epidemiology

Prognosis

The prognosis for patients with pyoderma gangrenosum is generally good; however, the disease may recur, and residual scarring is common. One study reported that 16% of their 103 patients died during the 8-year study period.^[8]Pain is a common patient complaint and may require pain medication for control.

Most patients with pyoderma gangrenosum improve with initial immunosuppressive therapy and require minimal care afterwards. However, many patients follow a refractory course, and multiple therapies may fail. These patients pose a difficult clinical problem that necessitates frequent follow-up and long-term care.

Some patients demonstrate pathergy, or the development of pyoderma gangrenosum–like lesions at the site of skin trauma; in such instances, protection of the skin from trauma may prevent a recurrence of the disease. Pathergy may create problems with wound healing, especially after surgical procedures (eg, breast reconstruction or grafting).^{[9, 10]}

Death from pyoderma gangrenosum is rare, but it may occur as a consequence of an associated disease or as a result of therapy.

Clinical Presentation

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Media Gallery

Classic, or typical, pyoderma gangrenosum. No associated disease was present, and condition responded well to cyclosporine.
Peristomal pyoderma gangrenosum.
Factitial ulceration on scalp from chronic manipulation, mimicking ulceration of pyoderma gangrenosum.

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Author

J Mark Jackson, MD Clinical Professor of Medicine/Dermatology, Division of Dermatology, University of Louisville School of Medicine

J Mark Jackson, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Kentucky Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Chief Editor

William D James, MD Emeritus Professor, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, American Contact Dermatitis Society, Association of Military Dermatologists, Association of Professors of Dermatology, American Dermatological Association, Women's Dermatologic Society, Medical Dermatology Society, Dermatology Foundation, Society for Investigative Dermatology, Washington DC Dermatological Society, Atlantic Dermatologic Society, Philadelphia Dermatological Society, Pennsylvania Academy of Dermatology, College of Physicians of Philadelphia

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Acknowledgements

David P Fivenson, MD Associate Director, St Joseph Mercy Hospital Dermatology Program, Ann Arbor, Michigan

David P Fivenson, MD is a member of the following medical societies: American Academy of Dermatology, Medical Dermatology Society, Michigan Dermatological Society, Michigan State Medical Society, Photomedicine Society, Society for Investigative Dermatology, and Wound Healing Society

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Michael J Wells, MD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Pyoderma Gangrenosum

Background

Epidemiology

Prognosis

References

Tables

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