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Ocular complications in 2 cases with porphyria
Ugur E Altiparmak1, Yusuf Oflu, Fatma Akbas Kocaoglu
1Ankara Training and Research Hospital, 1st Eye Clinic, Ankara, Turkey. ealtiparmak@hotmail.com
Cornea
|September 25, 2008
Summary
Ocular complications from porphyrias, like congenital erythropoietic porphyria (CEP) and porphyria cutanea tarda (PCT), can lead to severe vision loss. Even with intensive treatment, progressive scleral necrosis may occur, sometimes requiring eye removal.
Area of Science:
- Ophthalmology
- Genetics
- Dermatology
Background:
- Porphyrias are rare genetic disorders affecting heme biosynthesis.
- Ocular manifestations are infrequent but can be severe.
- Long-term follow-up data for ocular porphyria cases are limited.
Observation:
- Two patients with distinct porphyrias, congenital erythropoietic porphyria (CEP) and porphyria cutanea tarda (PCT), presented with similar ocular symptoms.
- Both experienced ocular pain, photophobia, and scleral necrosis.
- CEP patient had scleral necrosis with ectropion; PCT patient had nasal interpalpebral scleral necrosis.
Findings:
- Over a 4-year follow-up, both patients received intensive medical and surgical management, including amniotic membrane grafting.
- The PCT patient achieved a symptom-free outcome.
- The CEP patient experienced progressive scleral necrosis, ultimately requiring evisceration due to unresponsive endophthalmitis.
Implications:
- Scleral necrosis in porphyrias can be aggressive and lead to irreversible vision loss or eye loss.
- Aggressive management is crucial, but may not always prevent severe outcomes.
- Further research is needed to optimize the treatment of rare ocular complications in porphyria patients.
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