Ecthyma Gangrenosum 

  • Author: Mina Yassaee; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Nov 23, 2011
 

Background

Ecthyma gangrenosum (EG) is a well-recognized but uncommon cutaneous infection most often associated with a Pseudomonas aeruginosa bacteremia. EG usually occurs in patients who are critically ill and immunocompromised and is almost always a sign of pseudomonal sepsis. The characteristic lesions of EG are hemorrhagic pustules or infracted-appearing areas with surrounding erythema that evolve into necrotic ulcers surrounded by erythema. These were first described in association with Pseudomonas septicemia by Barker in 1897 and were later given the name "ecthyma gangrenosum" by Hitschmann and Kreibich. See the image below.

Violaceous plaques and necrotic ulcers on the abdoViolaceous plaques and necrotic ulcers on the abdomen of a renal transplant patient. Tissue cultures were positive for Pseudomonas aeruginosa.

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Pathophysiology

Impaired humoral or cellular immunity leads to increased susceptibility to infections with P aeruginosa or other pathogens. In addition, breakdown of mechanical defensive barriers, such as the skin and mucosa, may allow infectious organisms to disseminate. The lesions of EG result from perivascular bacterial invasion of arteries and veins in the dermis and subcutaneous tissues, producing a necrotizing vasculitis.[1] Perivascular involvement can occur by hematogenous seeding of the skin in bacteremic patients or by direct inoculation through the skin in nonbacteremic patients. Extravasation, edema, and necrosis around the vessel interrupt the blood supply to these tissues, resulting in secondary ischemic necrosis of the epidermis and dermis, which manifests as nodular lesions that rapidly evolve through stages of central hemorrhage, ulceration, and necrosis.

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Epidemiology

Frequency

United States

EG develops in 1.3-13% patients with P aeruginosa sepsis and, to a lesser extent, in patients who are not bacteremic.

Mortality/Morbidity

A high mortality rate is reported with delayed diagnosis and therapy. Mortality rates of Pseudomonas sepsis in immunocompromised persons range from 18-96%, whereas the mortality rate of EG in nonbacteremic patients is 15.4%. Coexisting conditions in patients prone to Pseudomonas sepsis may contribute to the morbidity and mortality rates.

Sex

No sexual predilection is evident in the overall prevalence of EG; however, a slight predominance of bacteremic EG in males (male-to-female ratio, 1.3-5:1) and nonbacteremic EG in females (female-to-male ratio, 2.3:1) has been observed.

Age

EG may affect patients of any age, although it is commonly reported in infants and elderly patients due to underdeveloped and/or compromised immune systems.

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Contributor Information and Disclosures
Author

Mina Yassaee  University of Pennsylvania School of Medicine

Mina Yassaee is a member of the following medical societies: Phi Beta Kappa and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Sarina Berger Elmariah, MD, PhD  Resident Physician, Robert O Perelman Department of Dermatology, New York University School of Medicine

Sarina Berger Elmariah, MD, PhD is a member of the following medical societies: Phi Beta Kappa

Disclosure: Nothing to disclose.

Ravi Ubriani, MD  Assistant Professor of Clinical Dermatology, Department of Dermatology, Columbia University Medical Center

Disclosure: Nothing to disclose.

William D James, MD  Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

Specialty Editor Board

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Christen M Mowad, MD  Associate Professor, Department of Dermatology, Geisinger Medical Center

Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors, Frederick Fish, MD, and Nobuyoshi Kageyama, MD, to the development and writing of this article.

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Violaceous plaques and necrotic ulcers on the abdomen of a renal transplant patient. Tissue cultures were positive for Pseudomonas aeruginosa.
 
 
 
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