Abstract

The porphyrias are a group of inherited disorders of heme synthesis causing an accumulation of porphyrins and / or their precursors leading to cutaneous photosensitivity and / or acute neurological symptoms. Ocular manifestations include blepharitis, lid abnormalities, sclerokeratitis, lipid keratopathy, scleromalacia, conjunctival and corneal ulceration and scarring. This is a case report of a 40 year old man with extensive cutaneous involvement for the past 5 years. He complained of mild irritation and redness in the eyes on and off relieved with tear substitutes. He had no vision complaints. On examination, in both eyes, he had pigmentary changes on the lid, normal lid and lash position, no congestion, ankyloblepharon laterally and an area of scleral melt close to the limbus temporally. In the left eye, the scleral melt was associated with ciliary staphyloma