PURPOSE: We aimed to report our observation regarding the long-term follow-up results of 2 cases with similar ophthalmic manifestations of 2 different porphyrias, congenital erythropoietic porphyria (CEP), and porphyria cutanea tarda (PCT). METHODS: Both patients presented with ocular pain and photophobia in both eyes. The patient with CEP had a scleral necrosis of 3 mm in diameter at the lateral limbus of the right eye and medial limbus of the left eye, accompanied with cicatricial ectropion and lid swelling OU. The patient with PCT had scleral necrosis in the interpalpebral area nasally, OU. RESULTS: Both patients were followed-up for 4 years. The patients received intensive topical lubrication and topical and oral immune-suppressive medication. They underwent amniotic membrane grafting, when required, and were advised to wear UV glasses. The case with PCT followed a more salient course and remained symptom free until the end of the follow-up period. In contrast, the patient with CEP developed further scleral necrosis, despite the treatment and evisceration surgery were inevitable owing to endophthalmitis unresponsive to the treatment. CONCLUSIONS: Ocular complications are rarely reported in porphyrias, and the studies on the long-term follow-up results are fewer. Despite careful follow-up and intensive treatment, scleral necrosis can be progressive and results in the loss of vision or even the loss of eye. Further studies regarding the care of patients with porphyrias are required to more effectively treat these rare ophthalmic conditions.
PURPOSE: We aimed to report our observation regarding the long-term follow-up results of 2 cases with similar ophthalmic manifestations of 2 different porphyrias, congenital erythropoietic porphyria (CEP), and porphyria cutanea tarda (PCT). METHODS: Both patients presented with ocular pain and photophobia in both eyes. The patient with CEP had a scleral necrosis of 3 mm in diameter at the lateral limbus of the right eye and medial limbus of the left eye, accompanied with cicatricial ectropion and lid swelling OU. The patient with PCT had scleral necrosis in the interpalpebral area nasally, OU. RESULTS: Both patients were followed-up for 4 years. The patients received intensive topical lubrication and topical and oral immune-suppressive medication. They underwent amniotic membrane grafting, when required, and were advised to wear UV glasses. The case with PCT followed a more salient course and remained symptom free until the end of the follow-up period. In contrast, the patient with CEP developed further scleral necrosis, despite the treatment and evisceration surgery were inevitable owing to endophthalmitis unresponsive to the treatment. CONCLUSIONS: Ocular complications are rarely reported in porphyrias, and the studies on the long-term follow-up results are fewer. Despite careful follow-up and intensive treatment, scleral necrosis can be progressive and results in the loss of vision or even the loss of eye. Further studies regarding the care of patients with porphyrias are required to more effectively treat these rare ophthalmic conditions.
Authors: Dulmini P Barupala; Stephen P Dzul; Pamela Jo Riggs-Gelasco; Timothy L Stemmler Journal: Arch Biochem Biophys Date: 2016-01-16 Impact factor: 4.013