Neurofibromatosis, von Recklinghausen's disease of the nervous system; analysis of the total pathologic picture.

Abstract

The PATHOLOGIC studies by von Recklinghausen covered a pleomorphic variety of disorders, but he is best known for the detailed description of two disease entities which bear his name. The first, frequently termed neurofibromatosis, or von Recklinghausen's disease, is characterized chiefly by a pigmentary and tumorous disorder of the skin, tumors of the nervous system and fibrocystic disease of the bones. The second, generalized osteitis fibrosa, characterized chiefly by fibrocystic disease of the bones, is wholly unrelated to the first disorder, with which it is sometimes confused. To make the distinction more definite, the term von Recklinghausen's neurofibromatosis, or von Recklinghausen's disease of the nervous system, has been used for the former, while von Recklinghausen's disease of bone designates the latter. The study of the morbid anatomic manifestations of von Recklinghausen's neurofibromatosis may be unusually difficult, not only because of the pleomorphism of the lesions but also because of the