Purpura Fulminans Due to Inherited Protein-C Deficiency in an Infant, in the Setting of MIS-C

• DOI: 10.4103/ipcares.ipcares_183_24
• Publication Date: 2025-02-14T13:00:46Z
• Authors: Anshika Gupta; Anu Maheshwari; Deonath Mahto; Mukesh Dhankar
• Keywords: covid-19; fresh frozen plasma; Pediatrics; RJ1-570; thrombophilia

Abstract

Background: Although multisystem inflammatory syndrome in children (MIS-C) may be associated with various mucocutaneous features, purpura fulminans (PF) has rarely been associated with it in children.

Clinical Description: A 10-month-old female infant presented with multiple ecchymotic patches on bilateral lower limbs and scalp gradually progressive over 5 months, associated with fever and irritability for 5 days. On examination, she was alert, hemodynamically stable, with severe pallor and corneal opacity in the left eye, along with the skin lesions, systemic examination being unremarkable.

Management and Outcome: Investigations revealed severe anemia, normal leukocyte, and platelet counts, elevated inflammatory markers, raised ferritin and NT pro-BNP levels. D-dimer was 5 times the normal range, COVID-19 real-time reverse transcriptase-polymerase chain reaction test was negative, but COVID-19 serology was positive >400 AU/ml. Echocardiography ruled out coronary dilatation. Ophthalmological evaluation revealed permanent hyperplastic vitreous in the left eye with retinal detachment in the right eye. The patient was managed along the lines of MIS-C with PF with broad-spectrum antibiotics, methylprednisolone, fresh frozen plasma, later adding cyclosporine, aspirin, and infliximab therapy. The baby showed remarkable improvement. Genetic analysis showed likely pathogenic heterozygous variant in the PROC gene which results in protein C deficiency.

Conclusion: This case highlights an inherited protein C deficiency manifesting as PF, in a setting of MIS-C in an infant. Prompt and aggressive management can be lifesaving.