1. Sight-threatening progressive corneo-scleral involvement in porphyria cutanea tarda
- Author
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Vidhata Vidhata, Sonali Prasad, and Subhash Prasad
- Subjects
Male ,Porphyria Cutanea Tarda ,medicine.medical_specialty ,genetic structures ,Conservative management ,Photophobia ,Eye Diseases ,Uroporphyrinogen III decarboxylase ,medicine ,Humans ,Uroporphyrinogen Decarboxylase ,Porphyria cutanea tarda ,skin and connective tissue diseases ,Serum ferritin ,medicine.diagnostic_test ,business.industry ,General Medicine ,Middle Aged ,medicine.disease ,Dermatology ,eye diseases ,Porphyria ,Serum iron ,sense organs ,medicine.symptom ,business ,Ocular surface ,Sclera - Abstract
Porphyria cutanea tarda is the most common type of porphyria. It is associated with a deficiency of uroporphyrinogen decarboxylase enzyme responsible for heme synthesis. Clinical manifestations are predominantly dermatological and very rarely present with ocular involvement. Although scleral thinning in the interpalpebral area is a well-documented entity, sight-threatening corneal involvement is rarely described. We, herein report a case of a 58-year-old man who presented with ocular surface dryness, photophobia and mild redness. Slit-lamp biomicroscopy revealed corneo-scleral thinning in both eyes. The diagnosis was confirmed with a urine porphyrin test, serum iron and serum ferritin levels. We started him on conservative management after which he was lost to follow-up. He presented again after 6 years with total corneal opacification and progressive loss of vision in the right eye.
- Published
- 2023