Ocular complications in 2 cases with porphyria.
OBJECTIVE
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.
Altiparmak
Ugur E
UE
Ankara Training and Research Hospital, 1st Eye Clinic, Ankara, Turkey. ealtiparmak@hotmail.com
Oflu
Yusuf
Y
Kocaoglu
Fatma Akbas
FA
Katircioglu
Yasemin Aslan
YA
Duman
Sunay
S
D000287:Administration, Topical; D000328:Adult; D000650:Amnion / Q000637:transplantation; D009877:Endophthalmitis / Q000209:etiology / Q000601:surgery; D005128:Eye Diseases / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D017234:Eye Evisceration; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D010146:Pain / Q000209:etiology*; D059408:Pain Management; D020795:Photophobia / Q000209:etiology* / Q000628:therapy; D017119:Porphyria Cutanea Tarda / Q000150:complications*; D017092:Porphyria, Erythropoietic / Q000150:complications*; D012590:Sclera / Q000473:pathology
D007166:Immunosuppressive Agents