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Stevens-johnson syndrome (dermatostomatitis; erythema multiforme exudativum; syndroma mucocutaneo-oculare; baader dermatostomatitis syndrome; mucosal-respiratory syndrome; fuchs [2] syndrome; mucocutaneous ocular syndrome)


Stevens-Johnson Syndrome (Dermatostomatitis; Erythema Multiforme Exudativum; Syndroma Mucocutaneo-Oculare; Baader Dermatostomatitis Syndrome; Mucosal-Respiratory Syndrome;
Fuchs [2] Syndrome; Mucocutaneous Ocular Syndrome)

General: Etiology unknown; affects all ages; most frequently seen in first and third decades of life; prevalent in males; drugs are the most commonly identified etiologic factor in this condition.

Ocular: Hypopyon; iritis; keratitis; corneal ulcers; keratoconjunctivitis sicca; chemosis; conjunctivitis; widespread fibrinoid necrosis of conjunctival vessels; blepharitis; endophthalmitis; phthisis bulbi; uveitis; cataracts; pannus; optic neuritis; keratoconus; adenoviral conjunctivitis has been reported to have precipitated Stevens-Johnson syndrome; orbital cyst may be a complication.

Clinical: General malaise, headaches, chills, and fever; severe skin and mucous membrane eruptions (erythema multiforme); dorsa of hands and feet are most frequently affected; rhinitis; balanitis; vulvovaginitis; urethritis (nonspecific); cystitis; patients with AIDS are at higher risk of developing Stevens-Johnson syndrome.

Arstikaitis MJ. Ocular aftermath of Stevens-Johnson syndrome. Arch Ophthalmol 1973; 90:376.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Geeraets WJ. Ocular Syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.

Gottschalk HR, Stone OJ. Stevens-Johnson syndrome from ophthalmic sulfonamide. Arch Dermatol 1976; 112: 513-514.

Hodge WG, et al. Adenoviral keratoconjunctivitis precipitating Stevens-Johnson syndrome. Can J Ophthalmol 1994; 29:198-200.

Kaido M, Goto E, Dogru M, et al. Punctal occlusion in the management of chronic Stevens-Johnson syndrome. Ophthalmology 2004; 111: 895-900.

Power WJ, et al. Analysis of the acute ophthalmic manifestations of the erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis disease spectrum. Ophthalmology 1995; 102:1669-l676.

Stevens AM, Johnson CF. A new eruptive fever associated with stomatitis and ophthalmia. Am J Dis Child 1922; 24:526.

Tabbara KF, Shammas HF. Bilateral corneal perforations in Stevens-Johnson syndrome. Can J Ophthalmol 1975; 10: 514-517.




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