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, [20] 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. [21, 22]
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 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. [23, 24]
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Violaceous plaques and necrotic ulcers on the abdomen of a renal transplant patient. Tissue cultures were positive for Pseudomonas aeruginosa.