Case of Von-Recklinghausen disease – A clinical report on the Diagnosis and Prosthodontic rehabilitation.

Abstract

Neurofibromatosis is a neurocutanous-skeletal syndrome caused by mutations in the NF1 tumor suppressor gene located at 17q11.2. It has a prevalence of ~ 1:2000–1:3000. The diagnosis of NF is based on criteria outlined by the National Institute of Health Consensus Development Conference in 1987. Cutaneous neurofibromas and café au lait spots are the hallmarks of NF1 on the skin. The oral involvement has been reported to occur in 3.4–92% of adult patients. Usually discrete, non-ulcerated nodules, varying from normal mucosal colour to red or even yellow, they are rarely seen occurring in 2% or possibly as many as 7% of cases. Since NF1 is not curable, diagnosis of such syndromes is of utmost importance such that proper and timely prosthodontic rehabilitation is offered to the patient for long term success of the prosthesis through multidisciplinary management. Dentists should be aware of the possible oral manifestations of neurofibromatosis because some of these lesions may become large enough to interfere with functions of mastication and speech. It is also imperative to put these patients on a long-term follow up for early detection of any possible signs of malignant transformation. Special attention should be paid to oral hygiene and regular follow up. This paper presents the diagnosis and prosthetic management for a case of Neurofibromatosis NF-1 in Recklinghausen disease.