MELANOSIS UVEAE AND MELANOMA OF THE IRIS IN NEUROFIBROMATOSIS (RECKLINGHAUSEN)

Abstract

Recklinghausen1in 1882 described melanoma in conjunction with multiple fibromas of the skin, the latter arising from cutaneous nerve filaments. It was soon found that fibromatous and the equally characteristic neurofibromatous lesions were not limited to peripheral nerves, but that such overgrowth could involve many portions of the nervous system. Moreover, the disease was related to a host of anomalies and associated disturbances, such as elephantiasis, pigmented plaques and small tumors of the skin, and in the nervous system to epilepsy, idiocy, defects in the special senses and endocrine disorders. For these reasons, Ewing2spoke of a congenital malformation of ectoderm leading to one or more manifestations under a variety of exciting causes. The orbit and the eye with its appendages have been found to exhibit these tendencies to a remarkable degree. Localized neurofibroma of the orbit and surrounding parts is fairly frequent. Less frequently, pigmented nevi