Idiopathic purpura fulminans associated with anti-protein S antibodies in children: a multicenter case series and systematic review

Abstract

Idiopathic purpura fulminans (IPF) is a rare but severe pro-thrombotic coagulation disorder that can occur after chickenpox or HHV6 infection. IPF leads to an autoantibody-mediated decrease in the protein S plasma concentration. We conducted a retrospective multicenter study involving IPF patients from 13 French pediatric centers and a systematic review of literature-published cases. Eighteen patients were included in our case series, and thirty-four as literature review cases. The median age was 4.9 years and the diagnostic delay after the first signs of viral infection was 7 days. The lower limbs were involved in 49 (94%) patients with typical lesions. A recent history of VZV or HHV6 infection was present in 41 (78%) and 7 (14%) of cases, respectively. Most of the patients received heparin (n=51, 98%) and fresh frozen plasma transfusions (n=41, 79%); other treatment options were immunoglobulin infusion, platelet transfusion, corticosteroid therapy, plasmapheresis, and coagulation regulator concentrate infusion. The antithrombin level and platelet count at diagnosis appeared to be associated with severe complications. Given the rarity of this disease, the creation of a prospective international registry is required to consolidate these findings.

Isolated Cephalohematoma as Presentation of Idiophatic Purpura Fulminans

“…IPF is a prothromobtic status, controversially anticoagulation should be used with alertness due to its increased bleeding risk expected for the CID situation. When thrombotic complications occur (large vessel or central venous catheter thrombosis), like in our case, it is recommended to start treatment with UHF 4 and close monitorization with anti-Xa should be performed. FFP should not be discontinued in this scenario.…”

“…It has been described in relation to infections such as Varicella, Streptococcus or herpes simplex type 6. [4][5][6][7] In our case, only a Rhinovirus and Enterovirus were found in a nasopharyngeal aspirate.…”

“…Haemorrhagic infarcts occur in necrotic lesions, with the formation of painful, indurated bullae. 4 Unlike purpura fulminans secondary to meningococcal sepsis, in which the affectation occurs in the distal part of the extremities, in idiopathic purpura fulminans the lesions tend to occur in other territories. Lower body affectation such as thighs, buttocks, calfs, scrotum and penis 4-6-7,10,11 have been described which generally occurs respecting distal part of extremities.…”

Post-viral idiopathic purpura fulminans is associated with inherited thrombophilia and anti-cardiolipin antibodies

“…This is an ancillary study of our French retrospective study and review of the literature ( 10 ). All the methodological details of the patient recruitment and data collection are detailed in this previous publication.…”

Evaluation of post-infectious inflammatory reactions in a retrospective study of 3 common invasive bacterial infections in pediatrics

“…[2,3] PIIR are usually attributed to established autoimmune mechanisms that are due to specific autoantibodies, such as acquired S protein deficiency following Varicella-Zoster Virus, and Human Herpes Virus-6 infection, with a putative breaking tolerance due to molecular mimicry between viral antigen and S protein. [4,5] Beyond these examples there are no robust data that confirm involvement of autoimmunity in most of PIIR.…”