Von Recklinghausen's Disease -Diagnosis from Oral Lesion. Neurofibromatosis I

The neurofibromatosis type 1 (NF1) was first described by Von Recklinghausen and Festscher, and has been known as the Von Recklinghausen’s disease also (Nagata, 2006). It is inherited as an autosomal dominant trait, therefore, is a hereditary condition. At least eight forms of neurofibromatosis have been recognized, but the most common is the (NF1), with a prevalence of 1:2200 to 3000 births (Friedman et al., 1999). About 50% of NF1 patients have no family history of the disease (Crowe et al., 1956; Riccardi, 1992). Also there is no prevalence for gender or race in NF1 (Bongiorno et al., 2006).

The signs and symptoms of this condition vary widely among affected people. The most common sign on the skin are multiple neurofibromas that can occur anywhere in the body. Another highly characteristic feature on the skin is the presence of café au lait (coffee with milk) pigmentation. Lisch nodules (pigmented hamartomas of the iris), translucent brown-pigmented spots on the iris, are found in nearly all affected individuals. Bone lesions, cardiovascular and neurological abnormalities are others manifestations of this disease (Alwan et al., 2005).