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April 25, 1931

CONGENITAL PLEXIFORM NEUROFIBROMAS AND ELEPHANTIASIS NEUROMATOSA OF THE RIGHT ARM AND NECK (VON RECKLINGHAUSEN'S DISEASE): SUPPLEMENTARY REPORT

Author Affiliations

Springfield, Ohio

JAMA. 1931;96(17):1381-1382. doi:10.1001/jama.1931.27220430002009a
Abstract

In 1919 I1 reported a case of von Recklinghausen's disease, with a review of the literature. The report may be thus summarized:

Francis S. appeared normal at birth. At the age of 1 year the boy cried when the upper part of the right arm was handled. The discomfort was due to subcutaneous fibrous cords occurring in the upper and lower parts of the arm. These were removed and the boy had considerable relief. In the course of years the whole right upper extremity grew in noticeable disproportion to that of the left arm. The skin became pigmented and thickened, simulating an ordinary elephantiasis. Brownish pigmentation gradually increased over the body in large areas. Roentgenograms of the bones of the right upper extremity showed trophic changes and atrophy.

To establish its congenital origin, it was shown that the father had multiple fibromas and pigmented spots over his body but

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