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STEVENS-JOHNSON SYNDROME A MIGHTY CHALLENGE TO MANAGE

BETSY CLEMENT

Abstract


Limbal stem cell deficiency is an ocular surface disease which can occur primarily or secondary to  inflammatory conditions such as Stevens-Johnson syndrome, ocular cicatricial pemphigoid, chemical or thermal burns.  Stevens-Johnson syndrome is a milder form of toxic    epidermal necrolysis which can have an idiopathic cause or may be triggered by infections or may be drug induced or malignancy related. These conditions require meticulous workup and treatment. Limbal stem cell transplantation is needed for the restoration of ocular surface and improvement of vision. The surgical treatment modalities available are transplantation of keratolimbal tissue, transplantation of  cultivated epithelial stem cells, amniotic membrane  transplantation, penetrating keratoplasty and  keratoprosthesis. The indication for keratoprosthesis is severe corneal disease with good visual potential and no  confounding factors such as glaucoma etc. Keratoprosthesis are used in patients with multiple failed grafts. Careful patient selection is needed in order to have beneficial outcome for the patient. The patient should be an adult, well motivated person, prepared for regular follow up fully aware of    associated risks of keratoprosthesis. This case brings out the challenges faced by both the doctor and the patient in   managing the sequelae of Stevens-Johnson syndrome. Only a team effort can achieve the goal of reconstructing a healthy ocular surface and providing useful to the patient.

 


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References


Epidermal apoptotic cell death in erythema multiforme and Stevens-Johnson syndrome. Contribution of perforin- positive cell infiltration. Arch Dermatol 1997; 133: 845-9

The natural history of Stevens Johnson syndrome: patterns of chronic ocular disease and the role of systemic immunosuppressive therapy.: Br J Ophthalmol. 2007 Aug; De Rojas

Keratoprosthesis: a 12-year follow-up. Girard LJ, Hawkins RS, Nieves R, rodofsky T, Grant C.


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