Iris melanocytoma mimicking the Cogan-Reese syndrome with monocular pigment dissemination.

PURPOSE: To report a case of iris melanocytoma mimicking the Cogan-Reese syndrome.

METHODS

A 37-year-old woman presented with pigmentary glaucoma in her left eye. There was diffuse pigmentary dispersion in the anterior segment, pedunculated pigmented nodules on the anterior iris surface, mild iris atrophy, and ectropion iridis. Neither intrinsic vasculature nor a sector cataract was found. The angle was open with marked trabecular pigmentation and no anterior synechiae. The intraocular pressure was 30 mmHg with maximum medical treatment and there was glaucomatous optic atrophy. The differential diagnosis included iris pigmented tumor and iridocorneal endothelial syndrome (Cogan-Reese syndrome). An iris biopsy was performed for diagnostic purposes.

RESULTS

Histologic diagnosis after evaluation of the specimen was iris melanocytoma.

CONCLUSIONS

This case presents signs considered quasi-pathognomonic of iridocorneal endothelial syndrome (Cogan-Reese syndrome): glaucoma, mild iris atrophy associated with pedunculated iris nodules, and ectropion iridis. Therefore, iris melanocytoma can present with features that mimic the Cogan-Reese syndrome.