Incontinentia pigmenti. Presentación neonatal.: A propósito de un caso clínico

La incontinentia pigmenti (IP) tambien conocida como sindrome de Bloch-Sulzberger, es una genodermatosis infrecuente ligada al cromosoma X que afecta tejidos derivados del neuroectodermo: piel, faneras, ojos, sistema nervioso central y dientes. En la etapa neonatal se plantean diagnosticos diferenciales como el impetigo ampollar, herpes neonatal, citomegalovirus, mastocitosis, epidermolisis ampollar hereditaria. El diagnostico temprano permite detectar las posibles patologias asociadas, que son determinantes para el pronostico del paciente.(AU) Incontinentia pigmenti, also known as Bloch-Sulzberger syndrome, is a rare congenital X-linked genodermatosis with variable involvement of tissues derived from neuroectoderm and mesoderm skin, hair, nails, eyes and central nervous system. Differential diagnoses are manifested in the neonatal period, such as bullous impetigo, neonatal herpes, cytomegalovirus, mastocytosis and hereditary epidermolysis bullosa. Early diagnosis allows detection of associated diseases which determine the patients prognosis.(AU)