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Picture of the Month—Quiz Case
Jon M. Burnham, MD;
Portia A. Kreiger, MD;
Michele Paessler, DO;
Leslie S. Kersun, MD;
Randy Q. Cron, MD, PhD
Author Affiliations: Divisions of Rheumatology (Drs Burnham and Cron) and Oncology (Dr Kersun), Department of Pediatrics, and Department of Anatomic Pathology (Drs Kreiger and Paessler), Children's Hospital of Philadelphia, Philadelphia, Pa.
Arch Pediatr Adolesc Med. 2006;160:1177.
An 11-year-old boy developed a generalized erythematous macular rash 5 weeks prior to hospitalization. The rash resolved in 3 weeks but he then developed fevers and a sore on his inner left arm (Figure 1). The lesion was painful and red. One week prior to hospital admission, a complete blood cell count revealed a low white blood cell count of 3.6 x 103/µL with a normal hemoglobin concentration and platelet count. A sore throat was present and he complained of oral ulcers.
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Figure 1. Painful, indurated left upper arm eschar with surrounding erythema.
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On hospital admission, he was ill appearing and febrile (40°C), with marked oral ulceration. In addition to an eschar measuring 1.5 cm on his left upper arm with underlying induration (Figure 1), he had a tender lymph node in the nearby axilla. His laboratory test results showed a white blood cell count of 1.6 x 103/µL (12% band forms, 67% segmented neutrophils, 20% lymphocytes), a hemoglobin concentration of 9.4 g/dL, and a platelet count of 71 x 103/µL. His fibrin split products were elevated and the fibrinogen level was falling. His serum ferritin level was 18 070 ng/mL (40 603 pmol/L). His C-reactive protein level was elevated at greater than 20 mg/dL, but surprisingly, his erythrocyte sedimentation rate was normal at 5 mm/h. His serum and urine electrolyte levels were consistent with syndrome-inappropriate antidiuretic hormone, and his uric acid and lactate dehydrogenase levels were elevated. Epstein-Barr virus testing was consistent with prior infection.
Biopsy specimens of the rash (Figure 2 and Figure 3) and a bone marrow aspirate (Figure 4) were diagnostic. Despite therapy for his underlying condition, the patient subsequently died of fungal sepsis.
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Figure 2 . Skin biopsy specimen revealing superficial necrosis and underlying atypical lymphoid infiltrate (hematoxylin-eosin, original magnification x 10).
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Figure 3. Higher-power view of skin biopsy specimen revealing atypical lymphoid infiltrate (hematoxylin-eosin, original magnification x 80).
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Figure 4. Bone marrow aspirate revealing hemophagocytosis.
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What is your diagnosis?
SECTION EDITOR: ALBERT C. YAN, MD; ASSISTANT SECTION EDITOR: SAMIR S. SHAH, MD
RELATED ARTICLE
Picture of the Month—Diagnosis
Arch Pediatr Adolesc Med. 2006;160(11):1178-1179.
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