Postinfectious purpura fulminans: A case report

Area of Science:

• Hematology
• Pediatric Medicine
• Dermatology

Background:

• Acquired postinfectious purpura fulminans is a rare, severe condition presenting with skin necrosis and disseminated intravascular coagulation (DIC).
• It predominantly affects children, often following an infectious episode, and is linked to transient autoantibody-mediated protein S deficiency.
• The underlying mechanism involves a temporary deficiency in protein S, leading to a hypercoagulable state.

Observation:

• A previously healthy 8-year-old boy presented with purpuric skin lesions characteristic of purpura fulminans.
• The patient exhibited signs of DIC without evidence of active sepsis.
• Diagnostic evaluation confirmed a transient plasma protein S deficiency.

Findings:

• The case demonstrates a clear association between purpura fulminans, DIC, and transient protein S deficiency in a pediatric patient.
• Treatment involved fresh frozen plasma replacement and anticoagulation therapy.
• Protein S activity remained diminished for two months post-event.

Implications:

• This case underscores the importance of recognizing transient protein S deficiency in the pathophysiology of acquired postinfectious purpura fulminans.
• Early diagnosis and appropriate management, including plasma replacement and anticoagulation, are crucial for favorable outcomes in affected children.
• Further research into the immunologic mechanisms of transient protein S deficiency may reveal novel therapeutic targets.