Lisch Nodules in Neurofibromatosis Type 2

Abstract

<h3>To the Editor.</h3> —Lisch nodules (pigmented iris hamartomas) are one of the diagnostic features of neurofibromatosis type 1 (NF-1, previously known as von Recklinghausen's disease or peripheral neurofibromatosis). The neurofibromatoses are a heterogeneous group of diseases but with two distinct forms: NF-1, characterized by multiple café au lait spots, peripheral neurofibromas, and Lisch nodules; and NF-2 (previously known as central neurofibromatosis), where café au lait spots and peripheral neurofibromas are absent or few, but the defining feature is the presence of bilateral acoustic neuromas. Currently accepted diagnostic criteria¹are more stringent than in the past, and it is now apparent that acoustic neuromas rarely occur in NF-1. Lisch nodules are found in 93% of adults with NF-1²but have not previously been described in NF-2. <h3>Report of a Case.</h3> —We recently examined a patient with NF-2 who had multiple Lisch nodules in one eye. He presented, aged 47