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Scleral Compromise in Hereditary Porphyria Cutanea Tarda
Eugenia Andrea Paez Soria1, Federico Andres Cremona1, Francisco Lucero Saá1
1Department of Ophthalmology, Hospital de Clínicas "José de San Martin", University of Buenos Aires, Buenos Aires, Argentina.
Journal of Current Ophthalmology
|June 4, 2021
Summary
Bilateral scleral thinning, a rare manifestation of porphyria cutanea tarda (PCT), can cause ocular discomfort. Early diagnosis and interdisciplinary management, including phlebotomy and hydroxychloroquine, can improve symptoms.
Area of Science:
- Ophthalmology
- Dermatology
- Genetics
Background:
- Porphyria cutanea tarda (PCT) is a metabolic disorder characterized by porphyrin accumulation.
- Ocular manifestations of PCT are uncommon, with scleral compromise being exceptionally rare.
Observation:
- A 57-year-old male with a history of PCT presented with bilateral scleral thinning, ocular discomfort, and photophobia.
- Clinical examination revealed characteristic skin findings of PCT, including hyperpigmentation, hypertrichosis, and scarring.
Findings:
- Diagnosis of PCT was confirmed through urine porphyrin testing and genetic analysis.
- Ocular findings included bilateral interpalpebral scleral thinning adjacent to hyperemic conjunctiva, without uveal prolapse.
Implications:
- This case highlights scleral thinning as a rare but significant ocular manifestation of PCT.
- Prompt diagnosis and multidisciplinary treatment, including systemic porphyrin reduction and photoprotection, are crucial for managing ocular symptoms in PCT patients.
Keywords:
Eye protective devicesPorphyriaPorphyria cutanea tardaPorphyria cutanea tarda/diagnosisScleral diseasesMore Related Videos
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