Background: Diffuse nodular nevus of the iris is an uncommon condition
that presents with multiple verrucous excrescences distributed diffus
ely on the iris surface. Methods: The authors describe 30 patients wit
h bilateral diffuse iris nodular nevi and report associations with bil
ateral congenital cataract, neurofibromatosis, oculodermal melanocytos
is, congenital ptosis, morning glory anomaly, Axenfeld anomaly, or Pet
ers anomaly. Results: Iris nodules were uniform in size and distributi
on and were brown, as was the surrounding iris, Light and electron mic
roscopy of iridectomy specimens from one patient showed elevated plaqu
es composed of aggregates of plump, lightly pigmented nevoid cells int
erwoven with mature, densely pigmented spindle-shaped uveal melanocyte
s. Conclusions: The authors report the largest clinical series and fir
st ultrastructural description of bilateral diffuse iris nodular nevi,
which represents a variant of neural crest development. No ocular com
plications could be attributed to the iris nodules, which should be di
fferentiated from Lisch nodules and other pathologic iris lesions.