Skip to main content
Log in

Varicella and thrombotic complications associated with transient protein C and protein S deficiencies in children

  • Original Paper
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

We report six cases of protein S deficiency secondary to varicella. Five cases were complicated by thrombotic and vascular events, namely purpura fulminans and necrotic vasculitis, deep vein thrombosis and stroke. Two cases were associated with protein C deficiency and one case revealed a heterozygous factor XII deficiency. The underlying mechanism of this acquired protein S deficiency is unclear but could be related to a direct effect of zoster virus.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

Abbreviations

APTT :

activated partial thromboplastin time

ATIII :

antithrombin III

DIC :

disseminated intravascular coagulation

PS:

protein S

IPG :

impedance plethysmography

PT:

prothrombin time

C4bBP :

C4b binding protein

References

  1. Andrew M, Paes B, Milner R, Johnston M, Mitchell L, Tollefsen DM, Powers P (1987) Development of the human coagulation system in the full-term infant. Blood 70:165–172

    Google Scholar 

  2. Auletta MJ, Headington JT (1988) Purpura fulminans. A cutaneous manifestation of severe protein S deficiency. Arch Dermatol 124:1387–1391

    Google Scholar 

  3. Comp PC, Nixon RR, Cooper MR, Esmon CT (1984) Familial protein S deficiency is associated with recurrent thrombosis. J Clin Invest 74:2082–2088

    Google Scholar 

  4. Comp PC, Elrod JP, Kerzneski IS (1990) Warfarin-induced skin necrosis. Semin Thromb Hemost 4:293–298

    Google Scholar 

  5. D'Angleo A, Della Valle P, Crippa L, Grimaldi L, Meschi F, Vigano d'Angelo S (1982) Severe thrombo-embolic disease due to autoimmune deficiency of vitamin K-dependent protein S. Thromb Res 65 [Suppl]:1 S 91

    Google Scholar 

  6. Dominey A, Kettler A, Yiannias J, Tschen JA (1989) Purpura fulminans and transient protein C and S deficiency. Arch Dermatol 124:1442–1443

    Google Scholar 

  7. Ducloux B, Martinot A, Leclerc F, Jude B (1989) Rôle du déficit acquis en protéine C dans une observation de thromboses artérielle pulmonaire et veineuses multiples. Pédiatrie 44:379–381

    Google Scholar 

  8. Francis RB (1990) Acquired purpura fulminans. Semin Thromb Hemost 4:310–325

    Google Scholar 

  9. Hessing M (1991) The interaction between complement component C4b-binding protein and the vitamin K-dependent protein S forms a link between blood coagulation and the complement system. Biochem J 277:581–592

    Google Scholar 

  10. Ozsoylu S, Cengiz B, Karabent A (1988) Purpura fulminans in a case of protein C deficiency. Eur J Pediatr 147:209–210

    Google Scholar 

  11. Ratnoff OD, Busce FJ, Sheon RP (1968) The demise of John Hageman. N Engl J Med 279:760–764

    Google Scholar 

  12. Seligsohn U, Berger A, Abend M, Rubin L, Attias D, Zivelin A, Rapaport S (1984) Homozygous protein C deficiency manifested by massive venous thrombosis in the newborn. N Engl J Med 310:559–562

    Google Scholar 

  13. Vukovich T, Auberger K, Weil J, Engelmann H, Knöbl P, Beat Hadorn H (1988) Replacement therapy for a homozygous protein C deficiency-state using a concentrate of human protein C and S. Br J Haemat 70:435–440

    Google Scholar 

  14. Yuen P, Cheung A, Ju Lin H, Ho F, Mimuro J, Yoshida N, Aoki N (1986) Purpura fulminans in a Chinese boy with congenital protein C deficiency. Pediatrics 77:670–676

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Nguyên, P., Munzer, M., Reynaud, J. et al. Varicella and thrombotic complications associated with transient protein C and protein S deficiencies in children. Eur J Pediatr 153, 646–649 (1994). https://doi.org/10.1007/BF02190684

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02190684

Key words

Navigation