Chickenpox is not always benign: postvaricella purpura fulminans requires prompt and aggressive treatment.
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.
Division of Paediatric Intensive Care, Children's University Hospital Berne, Berne, Switzerland.
Simon
S
Fluri
Gregor W
GW
Kaczala
Kurt
K
Leibundgut
Lorenzo
L
Alberio
D000956:Antigens, Viral / Q000276:immunology; D001327:Autoimmune Diseases / Q000209:etiology; D002644:Chickenpox / Q000150:complications*; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D004211:Disseminated Intravascular Coagulation / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology / Q000628:therapy; D005260:Female; D006562:Herpes Zoster / Q000276:immunology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D018448:Models, Immunological; D018716:Molecular Mimicry; D010949:Plasma; D011241:Prednisone / Q000627:therapeutic use; D017293:Protein S / Q000276:immunology; D018455:Protein S Deficiency / Q000175:diagnosis; D055665:Purpura Fulminans / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000628:therapy
D000956:Antigens, Viral; D016756:Immunoglobulins, Intravenous; D017293:Protein S; D011241:Prednisone