Posters

Bilateral and necrotizing scleritis in a late-onset cutaneous porphyria: case report

Poster Details

First Author: N.Boutimzine MOROCCO

Co Author(s):    K. Belkhadir   O. Cherkaoui   M. Hmidchat   A. Amazouzi   K. Senouci   R. Daoudi     

Abstract Details

Purpose:

Cutaneous porphyrias are rare metabolic disorders secondary to a deficiency in one of the enzymes involved in the synthesis of heme. Porphyria have in common cutaneous manifestations, whose primary is an acute or subacute photosensitivity. Ophthalmological manifestations are very rare, but can be severe and blinding. We report the case of a patient followed for sporadic late cutaneous porphyria with bilateral conjunctival and scleral necrosis.

Setting:

Ophtalmology Department A Ibn Sina University Hospital Rabat, Morocco

Methods:

A 42 years-old male patient, with a history of sporadic late-onset cutaneous porphyria, was hospitalized for a severe abscess of the cornea in the left eye. The physical examination revealed a central corneal abscess, occupying the entire corneal thickness, with hypopion. The examination of both eyes, showed several areas of scleral and conjunctival thinning and necrosis, located in the space between the eyelids, near the limbus and topped with thick yellowish plaques, embedded in the sclera. Bacteriological examination showed the presence of Staphylococcus epidermidis. The patient was successfully treated by fortified eye drops, associated with systemic antibiotics (ceftriaxone and moxifloxacin).

Results:

Three years later, the patient had a complication in his left eye: significant corneal thinning with descemetocele. The scleral plaque was removed, leaving a vascularized and thinned sclera area. Histological examination of this plate found necrotic tissue, partially calcified. A synthetic antimalarial treatment was initiated with a dermatological and ophthalmologic and biological monitoring.

Conclusions:

Ophthalmologic manifestations during porphyria are rare. Corneal ulcers and bilateral scleral necrosis can be found in late cutaneous porphyria, and are caused by phototoxicity due to the deposition of porphyrins’ precursors.

Financial Disclosure:

NONE