Arch Dermatol. 2009 Feb;145(2):157-62. LinkOut

Risk factors for the development of ocular complications of Stevens-Johnson syndrome and toxic epidermal necrolysis. Gueudry J, Roujeau JC, Binaghi M, Soubrane G, Muraine M. Department of Ophthalmology, Hôpital Charles Nicolle, 1 rue de Germont, 76031 Rouen, France. julie.gueudry@chu-rouen.fr

OBJECTIVES: To describe the acute and late ocular manifestations of toxic epidermal necrosis (TEN), Stevens-Johnson syndrome (SJS), and overlap syndrome and to identify predictors for the development of ocular complications.

DESIGN: Retrospective cohort study.

SETTING: A single referral unit in a university hospital.

PATIENTS: The study included 159 patients (mean [SD] age, 49.9 [19.8] years) with TEN and SJS during an 8-year period. Forty-nine patients were contacted at least 15 months after hospital discharge.

MAIN OUTCOME MEASURES: Records were reviewed for demographics, cause of the condition, and severity of ocular involvement. The patients were contacted to assess late ocular complications.

RESULTS: A total of 117 patients (74%) had acute ocular involvement, which was mild in 58%, moderate in 8%, and severe in 8%. Patients with TEN had more frequent (odds ratio [OR], 2.7; 95% confidence interval [CI], 1.06-6.90; P = .05) but not more severe (OR, 0.95; 95% CI, 0.20-4.5; P = .99) acute ocular involvement. Forty-nine patients were contacted at least 15 months after hospital discharge, and 63% had late ocular complications. Dry eye syndrome was the most common. The mean (SD) Ocular Surface Disease Index score was 32.9 (30.3) (range, 0-97.5). The severity of the acute ocular disease was found to be the only significant risk factor of late complications (P = .002), even though 5 patients without acute ocular involvement developed dry eye syndrome.

CONCLUSIONS: Ocular involvement is common in patients with SJS and TEN. Late complications are more frequent in patients with severe initial eye involvement but may also develop in patients without patent initial ocular symptoms.

Related Articles - Ocular manifestations and complications of Stevens-Johnson syndrome and toxic epidermal necrolysis: an Asian series. [Allergy. 2007] - Analysis of the acute ophthalmic manifestations of the erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis disease spectrum. [Ophthalmology. 1995] - Erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis: acute ocular manifestations, causes, and management. [Cornea. 2007] - ReviewHigh-dose intravenous immunoglobulin in the treatment of toxic epidermal necrolysis: a study of ocular benefits. [Eye. 2005] ReviewIntravenous immunoglobulin use in patients with toxic epidermal necrolysis and Stevens-Johnson syndrome. [Am J Clin Dermatol. 2006]

Ophthalmology. 2009 Feb 23. [Epub ahead of print] LinkOut

Diagnosis and Treatment of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis with Ocular Complications. Sotozono C, Ueta M, Koizumi N, Inatomi T, Shirakata Y, Ikezawa Z, Hashimoto K, Kinoshita S. Department of Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan.

PURPOSE: To present a detailed clarification of the symptoms at disease onset of Stevens-Johnson syndrome (SJS) and its more severe variant, toxic epidermal necrolysis (TEN), with ocular complications and to clarify the relationship between topical steroid use and visual prognosis.

DESIGN: Cross-sectional study.

PARTICIPANTS: Ninety-four patients with SJS and TEN with ocular complications.

METHODS: A structured interview, examination of the patient medical records, or both addressing clinical manifestations at disease onset were conducted for 94 patients seen at Kyoto Prefectural University of Medicine. Any topical steroid use during the first week at the acute stage also was investigated.

MAIN OUTCOME MEASURES: The incidence and the details of prodromal symptoms and the mucosal involvements and the relationship between topical steroid use and visual outcomes.

RESULTS: Common cold-like symptoms (general malaise, fever, sore throat, etc.) preceded skin eruptions in 75 cases, and extremely high fever accompanied disease onset in 86 cases. Acute conjunctivitis and oral and nail involvements were reported in all patients who remembered the details. Acute conjunctivitis occurred before the skin eruptions in 42 patients and simultaneously in 21 patients, whereas only 1 patient reported posteruption conjunctivitis. Visual outcomes were significantly better in the group receiving topical steroids compared with those of the no-treatment group (P<0.00001).

CONCLUSIONS: Acute conjunctivitis occurring before or simultaneously with skin eruptions accompanied by extremely high fever and oral and nail involvement indicate the initiation of SJS or TEN. Topical steroid treatment from disease onset seems to be important for the improvement of visual prognosis.

Related Articles -Analysis of the acute ophthalmic manifestations of the erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis disease spectrum. [Ophthalmology. 1995] Erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis: acute ocular manifestations, causes, and management. [Cornea. 2007] - Successful Treatment of Stevens-Johnson Syndrome with Steroid Pulse Therapy at Disease Onset. [Am J Ophthalmol. 2009] - ReviewChronic pulmonary complications associated with toxic epidermal necrolysis: report of a severe case with anti-Ro/SS-A and a review of the published work. [J Dermatol. 2006] - ReviewIntravenous immunoglobulin use in patients with toxic epidermal necrolysis and Stevens-Johnson syndrome. [Am J Clin Dermatol. 2006]

Am J Ophthalmol. 2009 Mar 13. [Epub ahead of print] LinkOut

Successful Treatment of Stevens-Johnson Syndrome with Steroid Pulse Therapy at Disease Onset. Araki Y, Sotozono C, Inatomi T, Ueta M, Yokoi N, Ueda E, Kishimoto S, Kinoshita S. Department of Ophthalmology, Kyoto Prefectural University of Medicine, Kyoto, Japan.

PURPOSE: To evaluate the visual prognosis of patients with Stevens-Johnson syndrome (SJS) and its severe variant, toxic epidermal necrolysis (TEN), followed by general and topical high-dose corticosteroids administration from disease onset.

DESIGN: Prospective, observational case series.

METHODS: Between May 1, 2003 and June 30, 2005, we enrolled 5 patients with SJS or TEN with ocular complications at the acute stage. Intravenous pulse therapy with methylprednisolone (steroid pulse therapy; 500 or 1000 mg/day for 3 to 4 days) was initiated within 4 days from disease onset. Topically, 0.1% betamethasone was applied over 5 times daily for at least 2 weeks. Visual acuity (VA) and slit-lamp microscopic appearance 1 year from disease onset were evaluated.

RESULTS: At the first examination, corneal or conjunctival epithelial defects and pseudomembranous conjunctivitis were present in all cases. Skin eruptions dramatically improved after steroid pulse therapy. Although ocular inflammation increased for several days, pseudomembranes disappeared and corneal and conjunctival epithelium regenerated within 6 weeks. At the chronic stage, all eyes had clear corneas with the palisades of Vogt (POV), implying the presence of corneal epithelial stem cells. Best-corrected VA was 20/20 or better in all eyes. Five eyes showed superficial punctate keratopathy. No eye had cicatricial changes except for 1 with slight fornix shortening. No significant adverse effects of steroid occurred during all clinical courses.

CONCLUSIONS: Steroid pulse therapy at disease onset is of great therapeutic importance in preventing ocular complications. Topical betamethasone also shows great promise for preventing corneal epithelial stem cell loss in the limbal region and cicatricial changes.

Related Articles - New grading system for the evaluation of chronic ocular manifestations in patients with Stevens-Johnson syndrome. [Ophthalmology. 2007] - Amniotic membrane with living related corneal limbal/conjunctival allograft for ocular surface reconstruction in Stevens-Johnson syndrome. [Arch Ophthalmol. 2003] - Penetrating keratoplasty in cicatrizing conjunctival diseases. [Ophthalmology. 1995] - ReviewVisual deterioration in giant cell arteritis patients while on high doses of corticosteroid therapy. [Ophthalmology. 2003] - Review[Clinical management of severe ocular surface disease] [Klin Monatsbl Augenheilkd. 2005] » See Reviews... | » See All... Patient Drug Information - Betamethasone Topical (Alphatrex® , Betatrex® , Beta-Val® , ...) Betamethasone is used to treat the itching, redness, dryness, crusting, scaling, inflammation, and discomfort of various skin conditions.