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March 1989

Multiple Cutaneous Plexiform Schwannomas With Tumors of the Central Nervous System

Author Affiliations

Department of Dermatology University of Genoa Viale Benedetto XV 16132 Genoa, Italy

Arch Dermatol. 1989;125(3):431-432. doi:10.1001/archderm.1989.01670150121022

To the Editor.—  Schwannomas (neurilemomas) are rare benign nerve tumors, usually presenting as solitary encapsulated subcutaneous nodules adherent to a peripheral nerve. It is most uncommon for schwannomas to possess multinodular intertwining characteristics (ie, a plexiform pattern). Plexiform schwannomas (PS) have often been confused with plexiform neurofibromas.1 Unlike the latter, which have a strong association with neurofibromatosis (NF), PS seem to affect exclusively the skin as solitary or multiple lesions.2,3 Nonetheless, there seems to be a small but definite association between PS and NF,2 and, recently, multiple cutaneous plexiform schwannomas (MCPS) have been reported in Japan as a marker of a novel neurocutaneous syndrome, neurilemomatosis (NL).4We report a case of MCPS with multiple tumors of the central nervous system in the absence of any cutaneous and genetic marker for NF.

Report of a Case.—  An 18-year-old normally intelligent woman was born to a family without

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