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Vol. 60. Issue 6.
Pages 585-588 (1 June 2004)
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Vol. 60. Issue 6.
Pages 585-588 (1 June 2004)
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Púrpura fulminante posvaricelosa
Postvaricella purpura fulminans
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L. Alsina Manrique de Laraa,
Corresponding author
cluaces@hsjdbcn.org

P.° Sant Joan de Déu, 2. 08950 Esplugues de Llobregat. Barcelona. España
, S. Zambudio Serta, A. Pizá Oliverasa, T. Toll Costab, J.J. García Garcíaa, C. Luaces Cubellsa
a Sección de Urgencias. Servicios de Pediatría Universitat de Barcelona. España
b Servicio de Hematología. Unidad Integrada Hospital Sant Joan de Déu-Clínic Universitat de Barcelona. España
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La púrpura fulminante es una complicación rara de la varicela que se caracteriza por la aparición progresiva de lesiones purpúricas o equimóticas dolorosas asociado a una alteración analítica propia de una coagulopatía de consumo. La activación de la coagulación es debida a un descenso marcado y prolongado de la proteína S, que probablemente es secundario a la formación de anticuerpos antiproteína S. El mecanismo responsable de la síntesis de estos autoanticuerpos es desconocido. Se presentan 3 casos de púrpura fulminante posvaricelosa y se revisan las características clinicoanalíticas y las recomendaciones diagnosticoterapéuticas actuales de esta entidad

Palabras clave:
Púrpurafulminans
Varicela
Proteína S
Anticuerpos antiproteína S

Purpura fulminans (PF) is an infrequent complication of varicella characterized by the progressive development of purpuric or painful ecchymotic lesions associated with biochemical alternations typical of consumption coagulopathy. Activation of coagulation is due to a marked and prolonged decrease in protein S, which is probably secondary to the formation of antiprotein S antibodies. The mechanism responsible for the synthesis of these autoantibodies is unknown. We present three cases of postvaricella PF and review the clinical and biochemical characteristics of this entity, as well as current diagnostic and therapeutic recommendations

Keywords:
Purpurafulminans
Varicella
Protein S
Antiprotein S antibodies
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Bibliografía
[1.]
E.G. Pérez-Yarza, L. Arranz, J. Alustiza, B. Azkunaga, J. Uriz, A. Sarasua.
y Grupo Varicela Gipuzkoa. Hospitalizaciones por complicaciones de la varicela en niños menores de 15 años.
An Pediatr (Barc), 59 (2003), pp. 229-233
[2.]
M. Levin, B.S. Eley, J. Louis, H. Cohen, L. Young, R.S. Heyderman.
Postinfectious purpura fulminans caused by an antibody directed against protein S.
J Pediatr, 127 (1995), pp. 355-363
[3.]
C.H. Van Ommen, M. Van Wijnen, F.G. De Groot, C.M. Van der Horst, M. Peters.
Postvaricella purpura fulminans caused by acquired protein S deficiency resulting from antiprotein S antibodies: Search for the epitopes.
Pediatr Hematol Oncol, 24 (2002), pp. 413-416
[4.]
G. Ángeles Fernández, J. Villanueva Lamas, F.J. Cambra, T. Toll Costa, A. Palomeque Rico.
Púrpura fulminans postvaricelosa con déficit transitorio de proteína C y proteína s.
An Esp Pediatr, 49 (1998), pp. 412-414
[5.]
T. Toll Costa, J. Estella Aguado, W. Youssef Fasheh, J. Bello Mayoraz.
Púrpura fulminans.
An Esp Pediatr, 36 (1992), pp. 481-483
[6.]
W.G. Phillips, J.R. Marsden, F.G. Hill.
Purpura fulminans due to protein S deficiency following chickenpox.
Br J Dermatol, 127 (1992), pp. 30-32
[7.]
J.r. Francis RB.
Acquired purpura fulminans.
Semin Thromb Hemost, 16 (1990), pp. 310-325
[8.]
F. Leclerc, J. Hazelzet, B. Jude, W. Hofhuis, V. Hue, A. Martinot, et al.
Protein C and S deficiency in severe infectious purpura of children: A collaborative study of 40 cases.
Intensive Care Med, 18 (1992), pp. 202-205
[9.]
D.M. Adrock, J. Brozna, R.A. Marlar.
Proposed classification and pathologic mechanisms of purpura fulminans and skin necrosis.
Semin Thromb Hemost, 16 (1990), pp. 333-340
[10.]
D. Borgel, S. Gandrille, M. Aiach.
Protein S deficiency.
Thromb Hemost, 78 (1997), pp. 351-356
[11.]
D.R. Powars, Z.R. Rogers, M.J. Patch, W.G. McGehee, R.B. Francis.
Purpura fulminans in menigococcaemia:association with acquired deficiencies of protein C and S.
N Engl J Med, 317 (1987), pp. 571-572
[12.]
M.J. Manco-Johnson, R. Nuss, N. Key, C. Moertel, L. Jacobson, S. Meech, et al.
Lupus anticoagulant and protein S deficiency in children with postvaricella purpura fulminans or thrombosis.
J Pediatr, 128 (1996), pp. 319-323
[13.]
P. Nguyen, J. Reynaud, P. Pouzol, M. Munzer, O. Richard, P. François.
Varicella and thrombotic complications associated with transient protein C and protein S deficiencies in children.
Eur J Pediatr, 153 (1994), pp. 646-649
[14.]
C. De Frutos Martínez, A. Iturrioz Mata, E. González Pérez-Yarza, M.a C. Arratibel Fuentes, R. Sainz Arroniz, Y. Albisu Andrade.
Púrpura fulminante idiopática con déficit transitorio de proteína.
An Esp Pediatr, 55 (2001), pp. 369-373
[15.]
C. Josephson, R. Russ, L. Jacobson, M.R. Hacker, J. Murphy, A. Weinberg, et al.
The varicella autoantibody syndrome.
Ped Res, 50 (2001), pp. 345-352
[16.]
Z. Kurugöl, F. Vardar, F. Özkinay, K. Kavakli, C. Özkinay.
Lupus anticoagulant and protein S deficiency in otherwise healthy children with acute varicella infection.
Acta Paediatr, 89 (2000), pp. 1186-1189
[17.]
M. Sorice, T. Griggi, P. Arcieri, A. Circella, F. d'Agostino, M. Ranieri, et al.
Protein S and HIV infection.
The role of anticardiolipin and antiprotein S antibodies. Thomb Res, 73 (1994), pp. 165-175
[18.]
C.R. Woods, C.A. Johnson.
Varicella purpura fulminans, associated with heterozygosity for factor V Leiden and transient protein S deficiency.
Pediatrics, 102 (1998), pp. 1208-1210
[19.]
S. Al-Ismail, P. Collins, R. Najib, M. James-Ellison, M. O'Hagan.
Postinfection purpura fulminans in a patient heterozygous for prothrombin G20210A and acquired protein S resistance.
Pediatr Hematol Oncol, 16 (1999), pp. 561-564
[20.]
P. Reinert.
Épidémiologie du purpura fulminans et recommandations thérapeutiques actuelles.
Arch Pédiatr, 8 (2001), pp. 673-676
[21.]
P.G. Hattersley.
Purpura fulminans: Complete recovery with intravenously administered heparin.
Am J Dis Child, 120 (1970), pp. 467-471
[22.]
N. Kuppermann, S. Hinkelis, R. Saladino.
The role of heparin in the prevention of extremity and digit necrosis in meningococcal purpura fulminans.
Pediatr Infect Dis J, 13 (1994), pp. 867-873
Copyright © 2004. Asociación Española de Pediatría
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