Ecthyma Gangrenosum Clinical Presentation

Updated: Jul 31, 2018
  • Author: Mina Yassaee Kingsbery, MD; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

Ecthyma gangrenosum (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 such as influenza B leading to a transient severe neutropenia, [16] 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. [17, 18]

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.

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Physical Examination

Primary lesions

Primary cutaneous lesions of ecthyma gangrenosum (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. [19, 20]

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