RESUMEN
Multisystem inflammatory syndrome in children (MIS-C) is a rare progressive inflammatory process temporally associated with exposure to SARS-CoV-2 (COVID-19) in patients 20 years of age and younger. At this time, much of MIS-C is not well understood, including the pathogenesis, long-term implications, and how each variant of the COVID-19 virus affects the progression and severity. We present the unusual case of a 19-year-old man with a history of homozygous sickle cell disease who developed a vaso-occlusive pain crisis and cerebral fat embolism syndrome as a complication of MIS-C secondary to the Omicron variant of COVID-19.
RESUMEN
A healthy 12-month-old female presented with relapsing and remitting urticaria since birth that was resistant to treatment with antihistamines. A thorough history revealed extensive rheumatic disease on the father's side of the family, and subsequent genetic testing was positive for a missense variant of NLRP3, indicating cryopyrin-associated periodic fever syndrome (CAPS). CAPS encompasses a spectrum of diseases, all related to a defect in the same gene; manifestations vary in severity and presentation, but most are associated with recurrent rash and fever. Because the patient's only presenting symptom was rash, this case highlights the importance of having a high index of suspicion for cryopyrin-associated periodic fever syndrome in infants with persistent, early urticaria.
