Generalized enchondromatosis associated with angiomas case report

ABSTRACT

The authors report a case of generalised enchondromatosis associated with a cutaneous angiomatosis realising the syndrome of MAFUCCI or type 11 of the classification of enchondromatosis of SPRANGER and A1. [1978]. This observation constitutes the first tunisian case. It is a rare entity, non familial, of unknown pathogeny discovered more often than not after the second year of life, because, of the shortening of lower limb or of the refraction of finger or a toe. The particular lesion of the disease is chondroma with often anarchic repartition about the skeleton. Its radiological aspect is that of homogeneous and transparent image with clear limits of oval or lengthened shape parrallel to the bone axis. The prognosis is reserved because of the evolutive tendances and the risk of degenerescence