We present 2 patients, who were admitted owing to rapidly progressing purpuric lesions due to postvaricella purpura fulminans, a coagulopathy leading to life- or limb-threatening thrombosis caused by a severe transient autoimmune protein S deficiency. Laboratory results were being consistent with disseminated intravascular coagulation secondary to protein S deficiency; treatment with fresh frozen plasma, intravenous immunoglobulins, and prednisone was started. In our experience, a prompt therapy may limit the course and the extent of the disease. We present a review of the topic with supporting literature for the therapeutic options. Therefore, we should be reminded that purpura fulminans is a rare but severe complication of chickenpox, which demands quick action.
From the *Division of Paediatric Intensive Care, Children's University Hospital Berne, Berne; †Division of Paediatric Intensive Care, Children's University Hospital Zurich, Zurich; ‡Division of Paediatric Haemato-Oncology, Children's University Hospital Berne; and §Department of Haematology and Central Haematology Laboratory, University Hospital Berne, Berne, Switzerland.
Reprints: Lorenzo Alberio, MD, Department of Haematology and Central Haematology Laboratory, University Hospital, Inselspital, 3010 Berne, Switzerland (e-mail: Lorenzo.firstname.lastname@example.org).
This study has no funding support.
The authors have no conflicts of interest.