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BACKGROUND
A 7-year-old girl is admitted to the hospital because of a 3-week history of fever, leukopenia, nausea, abdominal pain, and arm weakness. The patient had undergone a workup for infection at an outside facility prior to admission and was given a 5-day course of doxycycline to treat a fever of unknown cause. On this presentation, a peripheral blood smear is obtained and shows numerous leukoblasts. Subsequent bone marrow biopsy reveals acute lymphoblastic leukemia (ALL). Induction therapy, which includes dexamethasone, vincristine, pegylated (PEG)-asparaginase, and intrathecal methotrexate, is initiated.
Nine days after therapy begins, the patient has 2 tender, slightly raised, and erythematous lesions on her right arm. Her WBC count is 1.81 X 10/L (1810/µL) cm2 with a platelet count of 39 X 109/L (39 X 10/µL). The next morning, the largest lesion, which measures 15 mm, has a 5-mm darkened center. The lesions progress, and, by the next day, measure 20 mm with a 10-mm central area with dark discoloration (see Image 1).
Cefepime is administered for a suspected diagnosis of ecthyma gangrenosum, and fluconazole as prophylactic therapy is continued. Despite treatment with the antimicrobials, the lesion continues to grow and becomes increasingly tender to palpation, with progression to small surrounding satellite areas.
The patient is taken to an operating room, where the lesions are widely resected and samples are obtained to be sent to pathology and microbiology for examination. In addition to the tissue undergoing standard Gram staining and aerobic and anaerobic culture, acid-fast staining, mycobacterial culture, fungal staining, and viral culture are also performed.
Histopathology of the resected specimen reveals a 2.6 X 1.5 X 0.8-cm ellipse of skin that contains several lesions. The largest lesion is 1.0 X 1.3 cm and has a 1- to 2-mm rim of erythema around its dark center. About 4 mm from the largest lesion, a second lesion measures 3 X 2 mm and has surrounding erythema. The epidermis is laterally intact but centrally affected by ischemic necrosis (see Image 2). The dermis and subcutaneous tissues contain an extensive infiltrate of fungal hyphal elements involving the walls of the blood vessels, surrounding tissues, and focal perineural areas (see Image 3). Fungal elements are noted within 1 mm of each lateral resection margin (see Image 4).
What was the diagnosis?
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Hint
Areas of necrosis and fungal hyphae are noted during histopathology.
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Authors:
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Debra Whaley, MD,
Pediatric Resident,
Creighton University Medical Center,
University of Nebraska Medical Center,
Omaha, Neb
Meera Varman, MD,
Assistant Professor,
Pediatric Infectious Diseases,
Creighton University Medical Center,
Omaha, Neb
Chris Reyes, MD,
Department of Pathology,
Children's Hospital,
Adjunct Assistant Professor,
University of Nebraska Medical Center,
Omaha, Neb
Elizabeth Thompson, MD,
Professor,
Department of Pediatric Hematology-Oncology,
Children's Hospital,
Omaha, Neb
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eMedicine
Editor:
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Erik D. Schraga, MD,
Department of Emergency Medicine,
Kaiser Permanente,
Santa Clara Medical Center, Calif
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