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March 1989

SchwannomatosisAn Unusual Variant of Neurofibromatosis or a Distinct Clinical Entity?

Author Affiliations


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.

Arch Dermatol. 1989;125(3):390-393. doi:10.1001/archderm.1989.01670150080013

• 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)