[Basal cell XXXX syndrome. Presentation of 2 cases. 1 associated with @entity2946 ].

@entity1 @entity43 @entity101 @entity1276 @entity75 @entity5656 @entity5 @entity189 @entity7174 @entity17339 @entity2704 @entity4553 @entity6877 @entity74 @entity45 @entity2946 @entity729 @entity5766 @entity1491
A case of nevoid basal-cell @entity189 in a four years old @entity1 with preceding familiar history is reported. In her @entity189 father numerous basocellular, @entity45 , mandibular cysts, @entity74 , @entity75 ted and hiperkeratosis of the sole of the feet were described. Clinically, the @entity1 presented initial @entity2704 together with macrocephalus, bossing forehead, @entity5766 , @entity74 , retarded maturation, "keel" thorax, genu recurvatum, @entity4553 , hammerfinger, pigmented @entity7174 and @entity5656 of the sole of the feet. Radiologically @entity6877 of the skull suture with jagged image, endocranial @entity101 , signs of macrocephalus of the facial bones, @entity729 , @entity17339 and fusion of the ribs was observed. The axial computed tomography showed calcification of the vermix cerebelosus and severe @entity1276 suggesting the presence of an occupant mass. After total surgical removal of the mass, the histological examination revealed a @entity2946 . The skin biopsy of a cutaneous nevic element showed a @entity1491 . With the exception of an @entity43 and fusion of the vertebras non appreciable because of the age. With the exception of the @entity43 and the fusion of the vertebras, the @entity1 showed the same characteristics as those described in the classification of Gorlin. The importance of this paper communication lie on the preceding familiar history, infrequency, compromise of different systems, malignant tendency of the cutaneous lesions and frequent association with non cutaneous @entity5 .
