Fibromatose gingivale dans le cadre d'une atteinte syndromique

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 suggesting 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.