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  • Title: [Generalized congenital fibromatosis (author's transl)].
    Author: Larrègue M, Poitou C, Bressieux JP, de Giacomoni P, Vant F.
    Journal: Ann Dermatol Venereol; 1977 May; 104(5):349-54. PubMed ID: 921159.
    Abstract:
    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.
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