La enfermedad de Darier o disqueratosis folicular es una genodermatosis autosómica dominante. Se sabe que la variante segmentaria de la enfermedad, limitada a un segmento corporal y de la cual se conocen dos formas, es causada por un mosaicismo somático. Presentamos dos pacientes con enfermedad de Darier lineal con manifestaciones clínicas de la forma segmentaria tipo 1.
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