Purpura fulminans, presenting feature of multisystem inflammatory syndrome in children (MIS-C)

Abstract

Introduction. Coronavirus disease 2019 (COVID-19) has been a worldwide health issue since early 2020, caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Even though children do not usually present with se­vere COVID-19 unless the patient has an underlying di­sease, post­in­fectious complications, such as the multisystem in­flam­ma­to­ry syndrome in children (MIS-C), have been a sig­ni­fi­cant cause of child morbidity and mortality during the pan­de­mic. Even more challenging is that MIS-C has a very broad differential diagnosis due to its clinical heterogeneity. Case report. We report the case of a 6-month-old infant with­out another medical history who was brought to the ge­ne­ral practitioner’s office for persistent fever and cough. The symp­toms did not ameliorate under antipyretic and mucolytic treat­ment, so the patient later presented in the emergency room with associated dyspnea, diarrhea, vomiting and rapidly ge­ne­ra­li­zing purpuric skin lesions suggestive of purpura ful­mi­nans. He was admitted into our hospital and received em­pi­ri­cal antibiotic treatment. He also developed neck stif­fness. Meningococcemia was thus suspected; however, all cultures came out negative. Laboratory studies showed an important inflammatory syndrome, thrombocytopenia, se­vere anemia, coagulopathy, low protein C, protein S and an­ti­throm­bin levels, low fibrinogen, high D-dimer levels and hypo­al­bu­mi­ne­mia. Cerebral magnetic resonance imaging (MRI) showed no evidence of brain lesions. SARS-CoV-2 IgG-type antibodies were positive, therefore MIS-C was sus­pec­ted. The laboratory studies were extended and revealed high NT-proBNP, IL-6 and IL-2 soluble antigen levels. Other pos­si­ble diagnoses were excluded, which permitted our team to establish the diagnosis of MIS-C. The specific treatment was initiated with intravenous immunoglobulins (IVIG) and high-dose methylprednisolone associated with enoxaparin, broad-spectrum antibiotics and with symptomatic treatment. The patient rapidly evolved well, with the remission of fe­ver, inflammatory syndrome, coagulopathy and all other clinical and laboratory alterations. The skin lesions ame­lio­ra­ted as well, however, at a slower pace. Conclusions. MIS-C is a potentially life-threatening complication of COVID-19 in children. Therefore, clinicians must maintain a high degree of suspicion in the setting of the ongoing SARS-CoV-2 pandemic. However, the diagnostic criteria should be strictly followed due to the clinical similarities this pathology might have with other known diseases.