Diffuse but unilateral gingival enlargement associated with von Recklinghausen neurofibromatosis: a case report

An 8-year old girl was referred for diagnosis and treatment to the Department of Periodontology Oral Surgery of the University Hospital of Liège with an unusual clinical situation: a major, non-inflammatory, diffuse but unilateral enlargement of the interproximal, marginal and attached gingiva around all teeth of the right side of both the upper maxilla and mandible, whereas the alveolar process of the left side of upper and lower arches appeared strictly normal.

The clinical examination showed delayed eruption of some permanent teeth in the 1st and 4th quadrants. Except for its asymmetric occurrence, this gingival enlargement strongly resembled phenytoin-induced enlargement or gingival fibromatosis. This unilateral expression was evocative of a vascular or neurologic pathology. Several large "café-au-lait" spots were found disseminated on the body. Several selective surgical removals of thick gingival caps impairing the eruption of some permanent teeth were performed, and the removed tissues were histologically analyzed.

Because of the presence of the large "café-au-lait" spots, a clinical diagnosis of Von Recklinghausen's disease was given and later confirmed several times by the histological analysis of the gingival biopsies. Now, 6 years later, this gingival enlargement due to the development of intra-gingival neurofibromas is stable and all permanent teeth have had a normal eruption, but alveolar bone growth has been partly impaired by the presence of the tumor.

The present case of unilateral diffuse hyperplasia is a unique clinical expression of neurofibromatosis type I, a slowly evolving neurodermic dysplasia.