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Multicenter Study
. 2022 Jan 25;6(2):495-502.
doi: 10.1182/bloodadvances.2021005126.

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

Alexandre Theron  1   2 , Olivier Dautremay  3   4 , Elodie Boissier  3   5 , Amel Zerroukhi  1 , Julien Baleine  6 , Lionel Moulis  7 , Michel Rodière  8 , Jean-François Schved  2   3 , Martha Duraes  9 , Tarik Kanouni  10 , Isabelle Cau-Diaz  3 , Eric Jeziorski  8 , Christine Biron-Andreani  2   3
Affiliations
Multicenter Study

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

Alexandre Theron et al. Blood Adv. .

Abstract

Idiopathic purpura fulminans (IPF) is a rare but severe prothrombotic coagulation disorder that can occur after chickenpox or human herpesvirus 6 (HHV-6) infection. IPF leads to an autoantibody-mediated decrease in the plasma concentration of protein S. We conducted a retrospective multicenter study involving patients with IPF from 13 French pediatric centers and a systematic review of cases in published literature. Eighteen patients were included in our case series, and 34 patients were included 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 patients (94%) with typical lesions. In all, 41 patients (78%) had a recent history of varicella-zoster virus infection, and 7 patients (14%) had been infected by HHV-6. 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 seemed 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.

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Figures

None
Graphical abstract
Figure 1.
Figure 1.
Schematic depiction of typical lower limb lesions observed in many cases, with corresponding photographs of affected areas.
Figure 2.
Figure 2.
Box plot showing the age distribution, fibrinogen level, platelet count, antithrombin level, and PC activity at the time of diagnosis according to patient outcome (with or without amputation and/or skin graft). The box represents the interquartile (25th to 75th percentiles) range. The horizontal line in the box represents the median. The whiskers represent the maximum and minimum values (A) for case series patients and (B) for all patients (cases series and literatures review cases). The P value was estimated using Mann-Whitney nonparametric U test or Student t test. *P < .05; **P < .01; **P < .001.
Figure 3.
Figure 3.
Proposed algorithm for diagnostic and treatment of idiopathic fulminans purpura.
See this image and copyright information in PMC

References

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