Periorbital scleroderma associated with heterochromia iridis.

Two patients had biopsy-proven linear scleroderma, associated with a pigmentary abnormality of the anterior segment of the eye. One patient had heterochromia iridis, atrophy of the upper eyelid, a typical coup de sabre lesion, and a markedly asymmetric pigmentary glaucoma. The second patient had bilateral scalp involvement, paresis of the extraocular muscles, blepharoptosis, and heterochromia iridis.