Abstract

Neonatal-onset multisystem inflammatory disease (NOMID), also known as chronic infantile neurologic cutaneous articular syndrome, is progressive, and 20% of patients die before reaching adulthood. An urticarial rash develops in the first 6 weeks of life. Other problems ensue: a characteristic bony overgrowth, predominantly of the knees, and central nervous system abnormalities, including aseptic meningitis, increased intracranial pressure, cerebral atrophy, seizures, optic-nerve atrophy or papilledema, mental retardation, and sensorineural hearing loss. Hepatosplenomegaly and short stature are also possible outcomes. Laboratory findings include leukocytosis, …

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