Ictiosis ligada al x tratada con tazarotene tópico / X-linked ichthyosis treated with tazarotene topic

RESUMEN

La Ictiosis ligada al cromosoma X (ILX), es una genodermatosis poco frecuente que afecta a varones, se manifiesta a partir del período neonatal y se caracteriza por: xerodermia, hiperqueratosis, descamación y en algunos casos, manifestaciones extracutáneas. La delección total o parcial de la enzima sulfatasa esteroidea, es la causa de las manifestaciones clínicas. Presentamos el caso clínico, de un paciente con manifestaciones clínicas y hallazgos en la anatomía patológica, compatibles con esta entidad tratado con tazarotene (gel).

ABSTRACT

X-linked ichthyosis (XLI) is a rare genodermatosis that affects men and manifests from the neonatal period and is characterized by xeroderma, hyperkeratosis, desquamation and in some cases, extracutaneous manifestations. The disease is of chronic evolution and undergoes partial improvement in the summer season. The total or partial deletion of the steroid sulphatase enzyme is the cause of the clinical manifestations. We present the case of a patient with clinical manifestations and findings in the pathological anatomy compatible with this entity, as well as ITS treatment with topical tazarotene 0.1% (gel) Weekly controls were performed and clinical benefit of lesion-free skin was observed up to 6 weeks after treatment discontinuation, followed by the progressive appearance of brownish scales that became thick and adherent with the passage of time.