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An Unusual Variant of Neurofibromatosis or a Distinct Clinical Entity?

Maj Stephen M. Purcell, MC; Maj Steven L. Dixon, MC

Arch Dermatol. 1989;125(3):390-393.

Abstract
• Multiple schwannomas have frequently been seen in patients with neurofibromatosis. Recently, the association of multiple cutaneous schwannomas, central nervous system tumors, and various neurologic deficits has been described in Japanese patients as a condition called schwannomatosis. We describe the first non-Japanese cases of schwannomatosis and compare and contrast this unusual condition with the well-known variants of neurofibromatosis. We conclude that the features of schwannomatosis are distinct and define a condition that does not fit into the current classification scheme of neurofibromatosis. The occurrence of multiple cutaneous schwannomas in the absence of cardinal features of neurofibromatosis may indicate the presence of central nervous system tumors or various neurologic deficits.

(Arch Dermatol 1989;125:390-393)

Author Affiliations
USAF; USAF

From the Department of Dermatology, Wilford Hall US Air Force Medical Center, Lackland Air Force Base, Tex. Dr Purcell is now in private practice in Allentown, Pa.

Footnotes
Accepted for publication Oct 20, 1988.

Presented in part at the Gross and Microscopic Symposium of the American Academy of Dermatology, Dec 8,1985.

The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of Defense or the US Air Force.

Reprints not available.

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