Regresar

Incontinentia pigmenti or Bloch-Sulzberger syndrome

Abstract:

Incontinentia pigmenti, or Bloch-Sulzberger syndrome, is a rare dominant genodermatosis X-linked that affects almost exclusively to women; it is characterized by cutaneous, dental, vision and neurological damage. The typical skin symptoms of this condition consists of four stages: vesicular, verrucous, hyperpigmented and atrophic. This paper report two cases of pediatric patients in the Hospital Baca Ortiz, Quito, Ecuador, who had clear skin manifestations of the disease and so far no extracutaneous manifestations. This publication is important considering the rarity of this condition. Early diagnosis allows to identify any associated pathologies, which are decisive for the prognosis of the patient as well as to provide appropriate and timely treatment.