Background: Acquired purpura fulminans (APF) is a rare limb and life-threatening thrombotic disorder characterized by purpuric skin lesions and associated with deficiency of anticoagulant proteins C and/or S.

Aims: To describe the successful treatment of APF with protein C concentrate in a child and discuss incidence, risk factors, management, and outcomes of APF in children based on a comprehensive literature review.

Methods: Case details were obtained from patient records after ethics approval. For literature review, PubMed database was searched for articles from inception to 2020 using terms “acquired purpura fulminans” AND “children”.

Results: A 3-year-old previously healthy male was admitted to pediatric intensive care unit with septic shock and purpura on multiple digits. Protein C level of 0.16 U/mL (normal >0.70 U/mL) revealed the diagnosis of APF in the context of respiratory syncytial virus and S. aureus pneumonia. APF was initially managed with fresh frozen plasma (FFP), followed by protein C concentrate (Ceprotin) once available, to keep protein C level trough >0.50 U/mL, for 13 days. Patient was treated with concurrent enoxaparin for 3 months. The purpuric lesions started to improve on day 3-4, with complete resolution by 1 month. Patient recovered fully with no complications. The incidence of APF is 1 in 20,000 children. Risk factors include viral, bacterial (N. meningitidis being most common), or fungal infections as well as liver dysfunction, congenital heart disease, galactosemia, and antiphospholipid antibodies. Protein C replacement therapy, if started in a timely fashion (<24 hours from diagnosis), has been shown to reduce amputation rates and mortality in children with APF in retrospective studies. There are no prospective cohort or randomized controlled trials to date.

Pediatric Case Study of Purpura Fulminans: Lab Results Over The Disease Course

Conclusions: APF is important to recognize and manage promptly to prevent amputation and reduce mortality risk. Special access authorization required for protein C concentrate can delay initiation of treatment.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/a-case-report-and-comprehensive-review-of-acquired-purpura-fulminans-in-children/