From purpura to idiopathic purpura fulminans: a guidance in diagnosis and therapy

Abstract

Varicella is a common infection in children. Although benign in most cases, severe complications can occur, of which idiopathic purpura fulminans is one of the most devastating with high morbidity and mortality. It is a haematological emergency characterised clinically by well-demarcated erythematous maculae progressing rapidly to haemorrhagic necrosis. This form of purpura should be suspected in an otherwise healthy child with a history of varicella, no underlying sepsis, and coagulopathy with low protein S or C levels. Mimicking autoantibodies to anticoagulants, mainly against protein S, are thought to be aetiological factors. Therapy is based on the treatment of the underlying disease in combination with plasmapheresis and immunosuppression against the circulating autoantibodies. Depending on the degree of bleeding or hypercoagulability, plasma transfusion or anticoagulation may be considered.

We present a two-year-old girl with rapidly progressive purpura, leading to full-thickness skin necrosis requiring multiple surgeries and skin grafts. As early recognition and treatment is essential to reduce mortality and to prevent severe morbidity, awareness of this syndrome and its clinical evolution is important. We tried to establish a helpful guidance to facilitate diagnosis and treatment of idiopathic purpura fulminans.