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Case Reports
November 1932


Author Affiliations

Fellow in Pediatrics, the Mayo Foundation ROCHESTER, MINN.
From the Department of Pathology, University of Minnesota, and the Minneapolis General Hospital. Reported with permission of Dr. E. J. Huenekens.

Am J Dis Child. 1932;44(5):1040-1047. doi:10.1001/archpedi.1932.01950120122009

Von Recklinghausen's disease, multiple neurofibromatosis, is characterized by the formation of multiple connective tissue tumors on the nerve trunks, especially on the small cutaneous nerves.1 Pigmentation of the skin is almost constantly present in well developed cases. The first tumors to appear are usually those on the cutaneous nerves. Later tumors may develop on the larger and more deeply situated nerves. Tumors often develop on the intracranial portions of the cranial nerves and on the roots of the spinal nerves, giving rise to symptoms of tumor of the brain and tumor of the cord, respectively. Tumors developing on the sympathetic nerve trunks have been reported, but apparently they are rare in this situation.

The disease is frequently congenital, but the earliest manifestations may first appear in early childhood or at puberty. More frequently, the first symptoms develop in adult life. The condition occurs in successive generations, and appears to

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