[Generalized congenital fibromatosis (author's transl)].
About a spontaneously regressive case of osteo-cutaneous congenital fibromatosis, the authors describe the characteristics of the disease (32 observations). Although the majority of cases are sporadic, 3 familial observations are in favour of a dominant autosomal transmission of low penetrance. Study of the familial cases and analysis of the different localisations demonstrate the unicity of the so-called diffuse forms with visceral involvement and of the so-called generalized forms without visceral involvement congenital fibromatosis is characterized by several fibromas at birth: in two-thirds of the cases, it is a purely cutaneous or osteocutaneous form, which disappears spontaneously; in one third of the cases, it is a cutaneous or osteo-cutaneous form with lethal visceral involvement.
D001859:Bone Neoplasms / Q000151:congenital* / Q000175:diagnosis / Q000235:genetics; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005350:Fibroma / Q000151:congenital* / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D012878:Skin Neoplasms / Q000151:congenital* / Q000175:diagnosis / Q000235:genetics
Fibromatose congénitale généralisée.