Background

Pyoderma gangrenosum is an uncommon, ulcerative cutaneous condition of uncertain etiology. It is associated with systemic diseases in at least 50% of patients who are affected.^{[1, 2]}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.^{[3, 4]}Other organs systems that may be involved include the heart, the central nervous system, the gastrointestinal (GI) tract, the eyes,^{[5, 6]}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.)

Etiology

The etiology of pyoderma gangrenosum is poorly understood, but dysregulation of the immune system (specifically, altered neutrophil chemotaxis) is believed to be involved.

Epidemiology

Pyoderma gangrenosum occurs in about 1 in 100,000 persons each year in the United States. Although pyoderma gangrenosum affects both sexes, a slight female predominance may exist.^{[7, 8]}

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.)^[9]

Prognosis

The prognosis of 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 complaint of patients 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 requires 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, grafting).^{[10, 11]}

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

Clinical Presentation

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Media Gallery

Classic, or typical, pyoderma gangrenosum. This patient did not have an associated disease, and the condition responded well to cyclosporine.
Peristomal pyoderma gangrenosum.
Factitial ulceration on the scalp from chronic manipulation mimicking an 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 Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, 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, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<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.