Scleritis in congenital erythropoietic porphyria- infective
This case highlights the importance of awareness about the ocular complications in Congenital erythropoietic porphyria (CEP) that every general practitioner must be acquainted with and the fact that such a presentation should not be confused with an infective etiology.

CEP is an autosomal recessive disorder with deficiency of uroporphyrinogen III cosynthase. Ocular complications of CEP include nodular scleritis, necrotizing scleritis with inflammation or scleromalacia perforans. Necrotizing scleritis and scleromalacia perforans are among the most commonly reported ocular complications.

In the present case published in the Indian Journal of Ophthalmology, the patient had visual acuity of 6/12 in both eyes with scleritis, scleral thinning, and scleral calcific plaques evident on slit lamp examination; On systemic examination, patient had hypertrichosis, hyperpigmentation, depigmentation, fibrosis, and mutilation of ear, nose, and fingers.

Dental examination revealed brownish discoloration of teeth. Examination of hand and foot revealed acromiria, contracture of fingers, and atrophy of phalanges. Although the history and clinical findings suggested a possible diagnosis of scleritis with inflammation, the diagnosis was still missed by the primary treating physician.

CEP can be associated with devastating ocular complications. Necrotizing scleritis is one such complication, which if managed in time can avoid blinding outcomes as highlighted by dramatic healing in this case with topical prednisolone acetate, moxifloxacin, and lubricants at 1-week follow-up.

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