Scleral Necrosis in Porphyria Cutanea Tarda: A Case Report

Abstract

Scleral necrosis is a rare but well documented ocular manifestation of Porphyria Cutanea Tarda (PCT). The PCT is caused by a deficiency of Uroporphyrinogen Decarboxylase (Uro-D). The typical clinical manifestations of PCT in the form of cutaneous photosensitivity are due to the accumulation of fluorescent polycarboxylated porphyrins. Here, a case of 30-year-old male patient who was referred to the cornea services department for scleral thinning in right eye has been presented. Slit lamp biomicroscopy revealed focal area (5×5 mm2) of punched out scleral thinning (>80%) with uveal show in the interpalpebral area, 2 mm temporal to the limbus in right eye and a partial thickness scleral melt in nasal area. The left eye was phthisical. Patient had multiple blisters and pigmented, slightly depressed scars on the skin, especially in the sun exposed parts such as face, hands and legs. Digital shortening, atrophy and contractures were seen in hands and legs. Based on the clinical, biochemical and dermatological evaluation, the diagnosis of PCT was made. A rare case of scleral necrosis with uveal show in a patient with PCT which was successfully treated with allogenic scleral patch graft has been reported. On follow-up visits scleral patch graft was well taken up.

Keywords

• scleral patch graft
• scleral thinning
• uroporphyrinogen