Within the category of patients who have ocular surface disease is a special group of individuals who have been inflicted with Stevens-Johnson Syndrome (SJS). SJS is a rare condition that occurs most commonly in children or young to middle-aged adults. The onset of SJS is usually sudden and stormy with fever, malaise, sore throat, and respiratory symptoms. This is followed by the appearance of skin lesions of alternating red and white rings and bullae on the hands, feet, arms, legs, trunk, and face. The mucus membranes, especially of the eyes, mouth, and genitalia, may be particularly affected by these erosive lesions.

The etiology of SJS is unclear; however, it is most likely the result of an individual’s hyper-reactivity after exposure to any of a variety of substances or conditions that include systemic drugs (such as antibiotics and sulfonamides), respiratory infections, herpes simplex infection, or measles.

Ocular Manifestations

The mild form without mucosal involvement is actually erythema multiforme minor, which is a benign condition with no marked complications; previously, SJS was thought to be part of a clinical spectrum of disease that included erythema multiforme, SJS, and toxic epidermal necrolysis (TEN); however, according to Bastuji-Garin et al (1993), SJS/TEN is now considered a distinct entity. SJS inherently includes ocular involvement.

The ocular manifestations vary markedly and include severe sicca, conjunctival and corneal scarring, and keratinization of the ocular mucous membranes and lid margins. Other ocular manifestations include corneal vascularization, entropion, trichiasis, symblepharon, perforation, and blindness.

Not infrequently, the most serious long-term sequelae are the ocular complications, which often result in a lifetime of ocular surface management. Historically, SJS has been difficult for cornea specialists to manage due to patients’ ongoing sensitivity to traditional systemic and topical therapies; in many cases, patients become progressively worse regardless of the medical management. The management of the condition has improved with the introduction of modern scleral lenses.

A Case Study

One such patient is a 58-year-old male who suffered SJS at the age of five. At age 30, he was fitted with therapeutic soft contact lenses that were worn on a daily wear basis, with instillation of preservative-free artificial tears every 15 minutes and preservative-free ointment at night. Best-corrected visual acuity (BCVA) with the soft contact lenses was 20/60 in both eyes.

Seven years ago, we fitted him in scleral lenses, which he says “have changed his life forever” (Figures 1 and 2). Currently, he wears his sclerals 16 hours a day and is almost completely free of dry eye symptoms. His ocular surface has steadily improved throughout his years of wearing the scleral lenses, and today his BCVA is 20/25 in both eyes.

This is just one of thousands of patients who have severe ocular surface disease whose quality of life was dramatically changed with the introduction of modern scleral lenses. CLS

For references, please visit www.clspectrum.com/references and click on document #260.