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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.

Angiofibromas are firm, dome-shaped, skin-colored or pink-red, small papules (approximately 5 mm); they may be solitary or multiple. Solitary lesions on the dorsal aspect of the distal part of the nose in mature adults are called fibrous papules. However, multiple angiofibromas of the face, especially in a malar distribution, are typically associated with the tuberous sclerosis complex (TSC). Genetic linkage analysis has localized the defect in about one half of the cases to chromosome 9q (TSC1) and in the . . . [Full Text of this Article]

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