Facomatosis pigmentovascularis asociada a sndrome de Klippel-Trenaunay y a alopecia triangular. Reporte de caso / Pigmentovascularis phacomatosis associated to Klippel-Trenaunay syndrome and triangular alopecia. A case report

摘要

La facomatosis pigmentovascularis se caracteriza por la coexistencia de malformaciones vasculares cutneas con nevus melanocticos y hasta en un 50 % de los casos puede tener compromiso sistmico. El sistema de clasificacin tradicional identificaba cinco categoras, numeradas del I al V de acuerdo a la presencia de cierto tipo de nevus, subdivididos en tipo a y b dependiendo de si existe o no compromiso sistmico. Ms recientemente, Happle propone una nueva categorizacin basada en los hallazgos clnicos as: cesioflammea (mancha monglica y nevus flammeus), spilorosea (nevus de Spilus y nevus telangiectsico) y cesiomarmorata (mancha monglica y cutis marmorata telangiectsico congnita), siendo la primera la ms comn. Se presenta el caso de un paciente masculino de tres aos de edad, quien desde el nacimiento presentaba una malformacin vascular capilar tipo nevus flammeus extenso, mancha monglica, melanosis escleral e iris mamilado, una malformacin venosa congnita localizada en la regin suprapbica, adems de alopecia triangular, a quien se le hizo el diagnstico inicial de facomatosis pigmentovascularis tipo cesioflammea y posteriormente se asoci a sndrome de Klippel Trenaunay. ABSTRACT Phacomatosis pigmentovascularis is a disorder characterized by cutaneous vascular malformations associated with melanocytic nevi: up to 50 % of patients have systemic involvement. The traditional classification system identifies 5 categories numbered from I to V according to the presence of certain types of nevi, and it subdivides the categories into type a and b, depending on whether or not systemic involvement is present. More recently, Happle proposed a new classification based on clinical findings as follows: cesioflammea (Mongolian spot and nevus flammeus), spilorosea (telangiectatic nevus and nevus Spilus) and cesiomarmorata (Mongolian spot and congenital telangiectatic cutis marmorata), the first one being the most common one. We present a case of a 3 year old male patient who presented at birth with a capillary vascular malformation, an extensive nevus flammeus, a Mongolian spot, scleral melanosis and mammilated iris, a suprapubic congenital venous malformation and triangular alopecia; he was diagnosed with cesioflammea phacomatophacomatosis pigmentovascularis that was later associated with Klippel Trenaunay Syndrome.