Pyoderma Gangrenosum 

  • Author: J Mark Jackson, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Mar 23, 2010
 

Background

Pyoderma gangrenosum (PG) is an uncommon ulcerative cutaneous condition of uncertain etiology. Pyoderma gangrenosum was first described in 1930. It is associated with systemic diseases in at least 50% of patients who are affected. The diagnosis is made by excluding other causes of similar appearing cutaneous ulcerations, including infection, malignancy, vasculitis, collagen vascular diseases, diabetes, and trauma. Ulcerations of pyoderma gangrenosum may occur after trauma or injury to the skin in 30% of patients; this process is termed pathergy.

The 2 primary variants of pyoderma gangrenosum are the classic ulcerative form, usually observed on the legs, and a more superficial variant known as atypical pyoderma gangrenosum that tends to occur on the hands. Patients with pyoderma gangrenosum may have involvement of other organ systems that manifests as sterile neutrophilic abscesses. Culture-negative pulmonary infiltrates are the most common extracutaneous manifestation.[1] Other organs systems that may be involved include the heart, the central nervous system, the GI tract, the eyes,[2, 3] the liver, the spleen, bones, and lymph nodes. The prognosis of pyoderma gangrenosum is generally good; however, recurrences may occur and residual scarring is common. Therapy of pyoderma gangrenosum involves the use of anti-inflammatory agents, such as corticosteroids, and immunosuppressive agents.

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Pathophysiology

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

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Epidemiology

Frequency

United States

Pyoderma gangrenosum occurs in about 1 person per 100,000 people each year.

Mortality/Morbidity

Death from pyoderma gangrenosum is rare, but it may occur due to an associated disease or as the result of therapy. Pain is a usual complaint of patients and may require narcotics. Pathergy, or the development of pyoderma gangrenosum – like lesions at the site of skin trauma, may create problems with wound healing, especially after surgical procedures (eg, breast reconstruction).[4, 5]

Race

No racial predilection is apparent for pyoderma gangrenosum.

Sex

Pyoderma gangrenosum affects both sexes. A slight female predominance may exist.

Age

All ages may be affected, but pyoderma gangrenosum predominantly occurs in the fourth and fifth decades of life. Children may be affected, but they account for only 3-4% of the total number of cases. Pyoderma gangrenosum may affect children and adolescents. Nothing is clinically distinctive about these patients other than their age.[6]

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Contributor Information and Disclosures
Author

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

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

Disclosure: abbott, Amgen, Centocor Honoraria Speaking and teaching

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, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Specialty Editor Board

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.

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.

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.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

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Classic or typical pyoderma gangrenosum. This patient did not have an associated disease, and the condition responded well to cyclosporine.
Atypical pyoderma gangrenosum.
Peristomal pyoderma gangrenosum.
Factitial ulceration on scalp from chronic manipulation mimicking an ulceration of pyoderma gangrenosum.
 
 
 
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