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 × 103/μL with a normal hemoglobin concentration and platelet count. A sore throat was present and he complained of oral ulcers.
Painful, indurated left upper arm eschar with surrounding erythema.
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 × 103/μL (12% band forms, 67% segmented neutrophils, 20% lymphocytes), a hemoglobin concentration of 9.4 g/dL, and a platelet count of 71 × 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.
. Skin biopsy specimen revealing superficial necrosis and underlying atypical lymphoid infiltrate (hematoxylin-eosin, original magnification × 10).
Higher-power view of skin biopsy specimen revealing atypical lymphoid infiltrate (hematoxylin-eosin,
original magnification × 80).
Bone marrow aspirate revealing hemophagocytosis.
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