Fibromatose gingivale dans le cadre d’une atteinte syndromique

Abstract

Gingival fibromatosis is an inherited disease of the gingival tissue causing proliferative fibrous lesions occurring alone or as one feature of a more widespread syndrome. A 26-year-old girl consulted the oral surgery department for a functional problem due to the presence of two tumor masses located in the palate. The histological diagnosis was gingival fibromatosis. The presence of skeletal disorders in this patient with gingival fibromatosis suggested the diagnosis of Zimmermann Laband syndrome. Gingival fibromatosis is a rare condition characterized by a gingival hypertrophy. It is transmitted by autosomal dominant or recessive inheritance. The oral examination and histology provides the diagnosis. The treatment consistes of surgical removal of the hyperplasic fibrous tissue. Prognosis is dominated by the risk of recurrence which is frequent. Generally, gingival fibromatosis is isolated. In rare cases, it can be associated with symptoms sugges-ting a syndromic diagnosis such as Zimmermann Laband syndrome. In addition to gingival hypertrophy, skeletal anomalies can involve the fingers, the toenails, the thorax or the vertebrae. Splenomegaly and joint hyperextensibility can also be found.