• Claudia G Nevarez Flores, Alexander P Sun, and Howard Hast.
• Department of Pediatrics, Children's Hospital of Wisconsin Fox Valley, Neenah, Wisconsin; cnevarezflores@chw.org.
• Pediatrics. 2019 Jan 1; 143 (1).

AbstractPurpura fulminans is a rapidly progressive syndrome of intravascular thrombosis and hemorrhagic infarction of the skin. The most common infectious etiology is Neisseria meningitidis sepsis, and less commonly it has been documented as a complication of invasive Streptococcus pneumoniae In children who are otherwise healthy, splenic dysfunction is a significant predisposing factor for invasive pneumococcal infection. We present the case of a 10-month-old girl with a history of developmental delay, who developed an overwhelming infection complicated by purpura fulminans and was found to have previously undiagnosed Mowat-Wilson syndrome with anatomic asplenia. We propose screening patients with clinical features suggestive of Mowat-Wilson syndrome for asplenia to evaluate the need for additional preventive care.Copyright © 2019 by the American Academy of Pediatrics.

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