eMedicine Specialties > Dermatology > Bacterial Infections

Ecthyma Gangrenosum

Author: Mina Yassaee, University of Pennsylvania School of Medicine
Coauthor(s): Sarina Berger Elmariah, MD, PhD, Resident Physician, Robert O Perelman Department of Dermatology, New York University School of Medicine; Ravi Ubriani, MD, Assistant Professor of Clinical Dermatology, Department of Dermatology, Columbia University Medical Center; 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
Contributor Information and Disclosures

Updated: Jul 31, 2008

Introduction

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.

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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. 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.

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.

Clinical

History

  • EG typically occurs in patients who are immunocompromised, including patients with hematologic malignancies, immunodeficiency syndromes, severe burns, malnutrition, recent chemotherapy, immunosuppressive therapy, and diabetes mellitus. While a few case reports describe the development of EG in previously healthy children, most of these patients had unrecognized risk factors for the development of EG, including intra-abdominal or appendiceal abscesses, recent viral illness, or antibiotic treatment for underlying medical conditions such as hypogammaglobulinemia and neutropenia.
  • Two reports describe toxic epidermal necrolysis followed by EG, one in a 62-year-old woman and the other in a 3-year-old boy.1,2
  • Breakdown of mechanical defense barriers increases susceptibility to pseudomonal or fungal infections.
    • Pseudomonas sepsis frequently occurs after surgical procedures, especially urologic procedures.
    • Long-term indwelling urinary catheters, long-term intravenous placements, and tracheostomies have been associated with EG.
  • In several reported cases, patients with EG were on prolonged antibiotic therapy targeting non-Pseudomonas organisms. This may have led to elimination of normal flora and promoted Pseudomonas overgrowth.
  • Children with EG may develop diarrhea (30%) before the onset of cutaneous lesions.
  • Patients often present with fever a few days prior to developing EG.

Physical

  • Primary lesions: Primary cutaneous lesions of EG initially appear as painless round erythematous macules that rapidly become pustular with surrounding erythema. A hemorrhagic focus appears in the center, forming a bulla. As the hemorrhagic bulla spreads peripherally, it evolves into a gangrenous ulcer with a central black/gray eschar surrounded by an erythematous halo. The transformation of an early lesion to a necrotic ulcer may occur in as little as 12 hours.
  • Distribution of lesions: The patient may have a single lesion or multiple lesions. EG may appear at any location on the body; however, it predominately affects the anogenital and axillary areas. Distribution occurs at the following frequencies: gluteal or perineal region (57%), extremities (30%), trunk (6%), and face (6%); bilateral periorbital manifestations are rare but have been reported.3,4

Causes

EG is typically and most commonly caused by P aeruginosa; however, EG-like lesions have been observed in patients with other bacterial and fungal infections.5 Organisms that cause ecthyma and EG-like lesions include the following:

  • Gram-positive bacteria
    • Staphylococcus aureus
    • Streptococcus pyogenes
  • Gram-negative bacteria
    • Aeromonas hydrophila
    • Burkholderia cepacia6
    • Chromobacterium violaceum7
    • Citrobacter freundii
    • Corynebacterium diphtheriae
    • Escherichia coli
    • Klebsiella pneumoniae
    • Morganella morganii8
    • Neisseria gonorrhea
    • Pseudomonas aeruginosa
    • Pseudomonas stutzeri
    • Serratia marcescens
    • Vibrio vulnificus
    • Yersinia pestis
    • Xanthomonas maltophilia
  • Fungi
    • Aspergillus fumigatus
    • Candida albicans9
    • Curvularia species10
    • Exserohilum species
    • Fusarium solani11
    • Mucor and Rhizopus species
    • Pseudallescheria boydii10
    • Scytalidium dimidiatum
  • Viral - Herpes simplex virus12

More on Ecthyma Gangrenosum

Overview: Ecthyma Gangrenosum
Differential Diagnoses & Workup: Ecthyma Gangrenosum
Treatment & Medication: Ecthyma Gangrenosum
Follow-up: Ecthyma Gangrenosum
Multimedia: Ecthyma Gangrenosum
References
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References

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Further Reading

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Keywords

EG Pseudomonas aeruginosa, P aeruginosa, gram-negative infection, gram-negative bacteremia, immunosuppression, pseudomonal infection

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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; american college of physicians Honoraria Other

Medical Editor

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-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.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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.

Managing Editor

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.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine 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, 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.

 
 
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