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Abstract

Phakomatosis pigmentovascularis is a very rare disease characterized by coexistence of a capillary malformation with various melanocytic lesions, including dermal melanocytosis (Mongolian spots), nevus spilus, and nevus of Ota. As of now about 200 cases have been reported, most are of Japanese origin and about half of reported cases are associated with various systemic involvement. We present a 15-year-old Taiwanese male with phakomatosis cesioflammea who developed, during adolescence, a nevus spilus-like lesion and late-onset open angle glaucoma, suggesting that long-term ophthalmic follow-up is necessary in this type of patient.