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October 1952

NEUROFIBROMATOSIS: Report of Cases in Negro Subjects

Author Affiliations

From the Department of Pediatrics, Howard University, School of Medicine and the Pediatric Service of Freedmen's Hospital.

AMA Am J Dis Child. 1952;84(4):446-456. doi:10.1001/archpedi.1952.02050040054007

NEUROFIBROMATOSIS, often called von Recklinghausen's disease, is a well known clinical syndrome. Many articles on the subject have appeared in the literature, the report given by von Recklinghausen in 1882 being the best known.

The disease is a generalized congenital symptom complex involving predominantly the skin and nervous system1 and often associated with mental deficiency and skeletal defects. In addition, in some cases endocrinological dyscrasia and malignant transformation are exhibited. The condition shows a strong familial tendency.

In early infancy or childhood the disease may manifest itself in the abortive type, which consists of café-au-lait spots associated with freckles or hyperpigmentation. In the typical case in the older child and adult the condition often exhibits itself as tumors of the skin or molluscum fibrosum and deeper neural tumors.2 The five cases in this report tend to illustrate the multiplicity of findings in this syndrome.