Late onset of von Recklinghausen's disease

Neurofibromatosis is an autosomal dominant genetic disorder, although it may occur as a spontaneous mutation; clinically it is characterized by typical peripheral nerve tumours (Neurofibromas) and cafe au lait spots; they are more frequent over the trunk and legs. In this particular case study a patient with neurofibromatosis is described, who, besides the characteristic cafe au tait spots at birth, has developed, late and rapidly, lesions of neurofibromatous origin in the submammary region, right hip and bilaterally in the areolar region.