Neurofibromatosis type 2 (NF2) is an autosomal dominant disorder associated with bilateral vestibular schwannomas, schwannomas of other central and peripheral nerves, meningiomas, ependymomas, and opacities of the posterior capsular lens.1 This disorder results from the inactivation of a tumor suppressor gene (MERLIN) on chromosome 22q.2 Skin abnormalities associated with NF2 but more commonly seen in NF1 (von Recklinghausen disease) include neurofibromas and cafe au lait spots.
Jaffe AT, Heymann WR, Schnur RE. Clustered Angiofibromas on the Ear of a Patient With Neurofibromatosis Type 2. Arch Dermatol. 1998;134(6):760–761. doi:
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