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Esclerodermia lineal "en coup de sabre"
Patxi Zavala Gottau1, Francisco Caiza-Zambrano2, Luis Ariel Miquelini3
1Neurology Department, Hospital Britanico de Buenos Aires, Buenos Aires, Argentina patxizavalagottau@gmail.com.
Practical neurology
|January 23, 2026
Resumen
La esclerodermia lineal en coup de sabre, una afección rara de inicio en la edad adulta, puede manifestarse con problemas neurológicos como la epilepsia junto con lesiones en el cuero cabelludo. El diagnóstico y tratamiento tempranos con micofenolato mofetilo pueden estabilizar la afección.
Área de la Ciencia:
- Dermatología; Neurología; Patología
Sus antecedentes:
- La esclerodermia lineal en coup de sabre es una afección cutánea inflamatoria y fibrótica rara.; Infrecuentemente ocurre en adultos y raramente se presenta con manifestaciones extracutáneas, predominantemente neurológicas.
Objetivo del estudio:
- Informar un caso de esclerodermia lineal en coup de sabre de inicio en la edad adulta con afectación neurológica.; Destacar la importancia de considerar este diagnóstico en adultos que presentan lesiones en el cuero cabelludo y convulsiones.
Principales métodos:
- Presentación de caso de una mujer de 43 años con una lesión en la frente y epilepsia.; Imagen de resonancia magnética cerebral para evaluar cambios neurológicos.; Biopsia de piel para confirmación histopatológica de esclerodermia.
Principales resultados:
- La paciente presentó una lesión en la frente similar a una cicatriz y epilepsia.; La resonancia magnética cerebral reveló atrofia cortical focal ipsilateral y cambios en la sustancia blanca.; La biopsia de piel confirmó esclerosis hialina compatible con esclerodermia.; Se logró la estabilización clínica con micofenolato mofetilo.
Conclusiones:
- La esclerodermia lineal en coup de sabre de inicio en la edad adulta, aunque poco común, debe considerarse en adultos con lesiones en el cuero cabelludo y epilepsia.; Las manifestaciones neurológicas son significativas en los casos de inicio en la edad adulta.; El micofenolato mofetilo puede ser una opción de tratamiento eficaz.
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