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Esclerodermia - Overview

Nombres alternativos

Esclerosis sistémica progresiva; Esclerodermia limitada; Esclerosis sistémica; Síndrome de CREST; Esclerodermia localizada; Enfermedad mixta del tejido conectivo; Morfea lineal

Definición:

Es una enfermedad del tejido conjuntivo que involucra cambios en la piel, los vasos sanguíneos, los músculos y los órganos internos. Es un tipo de trastorno autoinmunitario, una afección que ocurre cuando el sistema inmunitario ataca por error y destruye tejido corporal sano.

Causas, incidencia y factores de riesgo:

Se desconoce la causa de la esclerodermia. Las personas con esta enfermedad presentan una acumulación de una sustancia llamada colágeno en la piel y otros órganos. Esta acumulación lleva a que se presenten síntomas de la enfermedad.

La enfermedad generalmente afecta a personas de 30 a 50 años de edad y las mujeres la desarrollan más a menudo que los hombres. Algunas personas con esclerodemia tienen antecedentes de estar alrededor del polvo de sílice y policloruro de vinilo, pero la mayoría no.

La esclerodemia generalizada puede ocurrir con otras enfermedades autoinmunitarias, entre ellas lupus eritematoso sistémico y polimiositis. En tales casos, el trastorno se denomina enfermedad mixta del tejido conjuntivo.

  • Reviewed last on: 2/14/2011
  • Michael E. Makover, MD, professor, attending in rheumatology, New York University Medical Center, New York, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Referencias

Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007.

Varga J, Denton CP. Systemic sclerosis and the scleroderma-spectrum disorders. In: Firestein GS, Budd RC, Harris ED Jr., et al., eds. Kelley's Textbook of Rheumatology. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2008:chap 77.

Clouse RE, Diamant NE. Esophageal motor and sensory function and motor disorders of the esophagus. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2006:chap 41.

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