The Unseen Emergency: Understanding Ocular Involvement in SJS/TEN

When we talk about Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN), the immediate focus is often on the severe and visible impact on the skin. However, SJS/TEN is a mucocutaneous condition, meaning it attacks the mucous membranes just as aggressively. For up to 80% of patients, the eyes are directly on the front lines of this immune response.

Because SJS/TEN is incredibly rare, bringing awareness to the different clinical topics within the disease is essential. Understanding the acute and long-term ocular impacts of SJS/TEN can help ensure patients get the specialized care they need from day one.

The Acute Phase: A Race Against Time

In the early days of an SJS/TEN reaction, ocular involvement might initially look like simple conjunctivitis (red, watery, and irritated eyes). However, it can rapidly progress to severe inflammation and blistering of the ocular surface.

During this acute phase, the same immune response causing the skin to detach is attacking one of the cell layers, called the epithelial layer, of the eyes. The greatest danger is the destruction of the limbal stem cells, which reside at the border of the cornea and the white of the eye (the conjunctiva). These cells are responsible for regenerating the clear surface of the eye; if they are destroyed, the eye loses its ability to heal properly.

The Game-Changing Treatment: Amniotic Membrane Transplantation

Because the damage happens so quickly, early intervention is critical. One of the most effective treatments for severe ocular involvement in the acute phase is amniotic membrane transplantation.

Ophthalmologists use cryopreserved amniotic membrane, which is tissue derived from the inner layer of a placenta, to cover the surface of the eyes and the inside of the eyelids. This membrane has powerful anti-inflammatory and anti-scarring properties. When applied within the first week or so of the reaction, it acts as a biological bandage, suppressing the inflammatory cascade and protecting the delicate tissues and stem cells from permanent scarring.

The Chronic Phase: A Lifelong Battle

For many survivors, the battle with SJS/TEN does not end when they leave the hospital or when their skin heals. Ocular complications can be chronic and require lifelong management.

Because the mucosal glands that produce tears are often damaged or destroyed, severe dry eye is common complication. In more severe cases, survivors may experience photophobia (extreme light sensitivity) or a condition called symblepharon, where the scarred inner eyelid fuses to the eyeball. Without ongoing specialized care, the ocular surface can become keratinized, which means it turns into hard, skin-like tissue to protect itself, and this can scratch the cornea and eventually lead to vision loss.

The Importance of Multidisciplinary Care

The complexity of ocular SJS/TEN highlights exactly why a multidisciplinary medical team is so crucial when managing this. An ophthalmologist, or eye [BC1] specialist, needs to be evaluating the patient on day one, not after the acute phase has passed. Amniotic membrane transplantation within the first week can potentially prevent severe, chronic complications. Following discharge, patients often require the long-term expertise of cornea specialists and specialized optometrists to fit them for scleral lenses, which help manage severe dry eye and protect vision.

References

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2.      Shanbhag SS, Chodosh J, Fathy C, Goverman J, Mitchell C, Saeed HN. Multidisciplinary care in Stevens-Johnson syndrome. Therapeutic Advances in Chronic Disease. 2020 Apr;11:2040622319894469.

3.      Saeed HN, Chodosh J. Ocular manifestations of Stevens–Johnson syndrome and their management. Current opinion in ophthalmology. 2016 Nov 1;27(6):522-9.

4.      Gregory DG. The ophthalmologic management of acute Stevens-Johnson syndrome. The ocular surface. 2008 Apr 1;6(2):87-95.

5.      Chang WC, Abe R, Anderson P, Anderson W, Ardern-Jones MR, Beachkofsky TM, Bellón T, Biala AK, Bouchard C, Cavalleri GL, Chapman N. SJS/TEN 2019: from science to translation. Journal of dermatological science. 2020 Apr 1;98(1):2-12.