Stevens-Johnson Syndrome

Stevens-Johnson syndrome (SJS) is a rare and potentially fatal autoimmune sensitivity reaction to infections or medications. It primarily affects the skin and mucous membranes, causing erosions and blisters as well as blindness in some people.

SJS is sometimes referred to as erythema multiforme major, which is a less severe form of the disease. At the other end of the spectrum, toxic epidermal necrolysis (TEN) is characterized by a confluence of blisters and erosions on more than 30% of the total body surface area.1 The incidence of SJS and TEN is approximately two cases per million persons per year.2

SJS can affect men, women and children of all ages, but it primarily occurs in young, healthy individuals, and it is more prevalent in females than males.3,4

The mortality rate for acute SJS ranges between 10% and 33%.5 As such, immediate medical intervention is typically aimed at saving lives—meaning secondary afflictions, including those involving the eye, are not always immediately addressed.6 The end result is often severe ocular problems that are notoriously challenging to address.

What Causes SJS?

Stevens-Johnson syndrome is a rare and unpredictable reaction. Your doctor may not be able to identify its exact cause, but usually the condition is triggered by a medication or an infection and is associated with widespread destruction of the epidermis.7 Several medications have been implicated in SJS, from common pain relievers to penicillin and more. Infectious causes likewise abound, with herpes and pneumonia as common, though not sole, culprits.

If your immune system is weakened, you may have an increased risk of developing SJS. A family history of the disease or the HLA-B 1502 gene likewise put you at increased risk.

Eye Care Needs for Patients with SJS

SJS commonly begins with flu-like symptoms that evolves into a rash that begins to spread and blister. These symptoms generally necessitate hospitalization and life-saving efforts. SJS can affect the eyes during this acute phase, or at some point down the road.8 ,9 In either case, interventions that address the ocular surface early on could make a big difference in visual outcomes later. For example, the inflammation of acute SJS can destroy limbal stem cells, leading to chronic issues with corneal health for the rest of a patient’s lifetime.

Late complications are more frequent in patients with severe initial eye involvement, but they can also develop in patients without patent initial ocular symptoms. 10 The most frequent late ocular complication is dry eye syndrome, which affects between 42% and 59% of patients. 11  Severe dry eye is a major concern because it can lead to worsening ocular surface health.12 Furthermore, such late ocular complications can significantly impair a patient’s overall quality of life. 13

Patients who have oular symptoms during the acute stage often experience inflammation. Severe ocular complications may even result in permanent visual loss due to corneal scarring or vascularization.14 Secondary infection also may develop in the acute stage.

Surgical and Pharmaceutical Treatments for SJS

Ophthalmic treatment options for SJS are determined by the severity of the condition and the effect it is having on the eyes. Medical eye care for SJS patients is directed towards improving ocular surface lubrication and decreasing inflammation.15  Such treatments include artificial tears, long-acting ocular lubricants, punctal occlusion, ophthalmic cyclosporine, topical corticosteroids, autologous serum eye drops, and moisture chamber eyewear. 16 However, these supportive treatments may be insufficient to improve the quality of life of afflicted individuals. 17 Also, since SJS is frequently initiated by a medication reaction, oral and topical medications should only be prescribed after thoughtful consideration.

Ophthalmic surgery is also often needed for SJS patients. In such cases, reconstructive surgery, grafting procedures, and stem cell transplantation may be considered.

Scleral Lenses for SJS

Scleral lenses have several benefits for SJS patients and are used in a couple of different ways. For some SJS patients, the lenses are worn overnight to help protect the corneal surface.1819 Indeed, research has shown scleral lens use to be efficient and safe for visual rehabilitation of refractory ocular surface disease attributable to SJS.20

These uniquely designed lenses vault across the entire corneal surface and rest on the white part of the eye, known as the sclera. A liquid reservoir is formed between the lens and the cornea, which can be instrumental in protecting the cornea from further scarring.

Scleral lenses are also used by SJS patients following an acute SJS event. Because SJS causes dry eye and often leads to trichiasis (inward turning of the eyelashes) or keratinization (hardening and callousing) of the lid margin, both situations that make blinking extremely uncomfortable. The mechanical shearing effect of the eyelashes and eyelids, respectively, can feel like sandpaper. Scleral lenses aim to reduce this sensation by creating a protective barrier over the front surface of the eye, while continuously bathing the sensitive corneal tissue.

If you have SJS, it is critical to see your eye doctor as regularly as recommended so you can be closely monitored for a recurrence and for any future ocular surface damage.

 


[1] Gueudry J, Roujeau J, Binaghi M, Soubrane G, Muraine M. Risk Factors for the Development of Ocular Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Arch Dermatol. 2009;145(2):157-162.

[2] Rzany  BMockenhaupt  MBaur  S  et al.  Epidemiology of erythema exsudativum multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis in Germany (1990-1992): structure and results of a population-based registry. J Clin Epidemiol 1996;49 (7) 769- 773

[3] Kanski JJ. Stevens-Johnson syndrome. In: Clinical Ophthalmology. A Systemic Approach. 3rd ed. Oxford, England: Butterworth-Heinemann Ltd. 1994:89.

[4] Kunimoto DY, Danitkar KD, Makar MS, et al. Stevens-Johnson syndrome. In: The Wills Eye Manual. 4th ed. Philadelphia: Lippincott Williams & Wilkins. 2004:344-5.

[5] Kunimoto DY, Danitkar KD, Makar MS, et al. Stevens-Johnson syndrome. In: The Wills Eye Manual. 4th ed. Philadelphia: Lippincott Williams & Wilkins. 2004:344-5.

[6] Dalton M. Newer treatments offer hope for Stevens-Johnson Syndrome. EyeWorld. March 2010.

[7] Tougeron-Brousseau B, et al. Vision-Related Function After Scleral Lens Fitting in Ocular Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. American Journal of Ophthalmology. 148.6 (Dec 2009): 852-859.

[8] Tougeron-Brousseau B, et al. Vision-Related Function After Scleral Lens Fitting in Ocular Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. American Journal of Ophthalmology. 148.6 (Dec 2009): 852-859.

[9] Gueudry J, Roujeau J, Binaghi M, Soubrane G, Muraine M. Risk Factors for the Development of Ocular Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Arch Dermatol. 2009;145(2):157-162.

[10] Gueudry J, Roujeau J, Binaghi M, Soubrane G, Muraine M. Risk Factors for the Development of Ocular Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Arch Dermatol. 2009;145(2):157-162.

[11] Tougeron-Brousseau B, et al. Vision-Related Function After Scleral Lens Fitting in Ocular Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. American Journal of Ophthalmology. 148.6 (Dec 2009): 852-859.

[12] Nakamura T, Inatomi T, Sotozono C, et al. Transplantation of cultivated autologous oral mucosal epithelial cells in patients with severe ocular surface disorders. Br J Ophthalmol 2004 Oct;88(10):1280-4.

[13] Tougeron-Brousseau B, et al. Vision-Related Function After Scleral Lens Fitting in Ocular Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. American Journal of Ophthalmology. 148.6 (Dec 2009): 852-859.

[14] Sotozono  CAng  LPKoizumi  N  et al.  New grading system for the evaluation of chronic ocular manifestations in patients with Stevens-Johnson syndrome. Ophthalmology 2007;114 (7) 1294- 1302

[15] Tougeron-Brousseau B, et al. Vision-Related Function After Scleral Lens Fitting in Ocular Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. American Journal of Ophthalmology. 148.6 (Dec 2009): 852-859.

[16] Tougeron-Brousseau B, et al. Vision-Related Function After Scleral Lens Fitting in Ocular Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. American Journal of Ophthalmology. 148.6 (Dec 2009): 852-859.

[17] Tougeron-Brousseau B, et al. Vision-Related Function After Scleral Lens Fitting in Ocular Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. American Journal of Ophthalmology. 148.6 (Dec 2009): 852-859.

[18] Fine P, Savrinski B, Millodot M. Contact lens management of a case of Stevens-Johnson syndrome: a case report. Optometry 2003 Oct;74(10):659-64.

[19] Tappin MJ, Pullum KW, Buckley RJ. Scleral contact lenses for overnight wear in the management of ocular surface disorders. Eye 2001 Apr;15(pt 2):168-72.

[20] Tougeron-Brousseau B, et al. Vision-Related Function After Scleral Lens Fitting in Ocular Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. American Journal of Ophthalmology. 148.6 (Dec 2009): 852-859.