Background: An 18-month-old female toddler presented with severe vulvar ulcers and pancytopenia with investigations revealing Pseudomonas aeruginosa bacteremia.
Case: A previously healthy 18-month-old female toddler presented with 6 days of fevers, vulvar rash, and ulcers. Vulvar cultures showed Staphylococcus aureus and P aeruginosa. Bloodwork showed pancytopenia and P aeruginosa bacteremia. She started receiving broad-spectrum antibiotics. Bone marrow aspirate revealed a hypocellular marrow with erythroid dysplasia. Vulvar ulcers progressed rapidly, therefore magnetic resonance imaging was performed to rule out necrotizing fasciitis. She was diagnosed with ecthyma gangrenosum (EG). Three months after initial presentation, she was diagnosed with precursor B-cell acute lymphoblastic leukemia.
Summary and conclusion: This case highlights that health care providers should suspect EG when severe vulvar ulcers are present with P aeruginosa infection and neutropenia. Because EG poses significant morbidity and mortality, its presence should prompt aggressive antimicrobial therapy and mobilization of a multidisciplinary team to initiate workup for an underlying immunodeficiency syndrome or malignancy. This case also illustrates that surgical debridement might be avoided in certain patients with EG as long as meticulous wound care and close monitoring with a multidisciplinary team are in place.
Keywords: Acute lymphoblastic leukemia; Ecthyma gangrenosum; Pseudomonas aeruginosa; Toddler; Vulvar ulcers.
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