Observation:
We report here a rare case of Neurofibromatosis Type 1 (NF1) in a 42 -year-old female who developed Behçet’s disease (BD). NF1 was diagnosed twenty years ago and BD was diagnosed at 42 years of age. NF1 is an autosomal dominant disease with an incidence of 1 in 2,600 to 3,000 individuals. BD is a systemic vasculitis with obscure etiology, characterized by recurrent episodes of oral and genital ulcers, skin lesions, and ocular lesions. Although there are a few reports on the combination of Incontinentia Pigmenti and BD, this is the first reported case that concurrence of NF1 with Plexiform neurofibrom and BD.
Keywords: Behçet’s Disease, plexiform neurofibroma, neurofibromatosis