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2012, Número 3

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Gac Med Mex 2012; 148 (3)


Síndrome de Stevens-Johnson y necrólisis epidérmica tóxica en los niños

Sotelo-Cruz N

Idioma: Español
Referencias bibliográficas: 51
Paginas: 265-275
Archivo PDF: 103.06 Kb.


RESUMEN

El síndrome de Stevens-Johnson (SSJ) y la necrólisis epidérmica tóxica (NET), manifestados en la mayoría de los casos como una reacción cutánea a medicamentos, constituyen un mismo espectro clínico, con diferente grado de epidermólisis; ambas entidades se distinguen por la severidad y extensión de las lesiones en piel; pueden afectarse, además, mucosas, ojos, vías respiratorias, sistema digestivo y tracto urogenital; el evento ocurre anualmente en dos casos/1,000,000; en los niños se presenta en aproximadamente el 20% del total, y ambas son consideradas entidades potencialmente mortales. Hasta el momento actual la fisiopatología exacta no ha sido completamente aclarada, no obstante que el SSJ fue identificado hace 89 años. En su génesis se invoca un mecanismo mediado inmunológicamente, que involucra linfocitos citotóxicos, citocinas y Fas-ligandos en la apoptosis de queratinocitos; se han identificado también, en algunos grupos raciales, marcadores genéticos (antígeno leucocitario humano [HLA]-B*1502, HLA-B*5801), relacionados con susceptibilidad específica a algunos fármacos como carbamacepinas y alopurinol. En niños no existen criterios uniformes de clasificación para severidad de las lesiones, ni para el tratamiento, no obstante recientemente los autores coindicen en mejor evolución de los pacientes con el uso de inmunoglobulina intravenosa.


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