1. What is Steven ¬Johnson Syndrome (SJS)?
Stevens-Johnson syndrome (also called erythema multiforme major) is a rare condition arising from ‘over-reaction’ of the immune system due to a trigger (such as a mild infection or a medicine or vaccine), leading to blistering and peeling of the skin and surfaces of the eyes, mouth and throat. It is named after the two doctors who described it.
SJS occurs twice as often in men as in women, and most often affects children and young adults under 30, although it can develop at any age.
2. What are the signs and symptoms of SJS in the eye?
The acute stage of the SJS is characterized by bilateral catarrhal and membranous conjunctivitis. During the chronic stage of the disease, most patients have various alterations of the ocular surface, such as symblepheron, entropion, trichiasis, dry eye, limbal stem cell deficiency, conjunctival inflammation and corneal neovascularization.
3. What is the medical management of SJS?
While there is no standard treatment, a combination of topical corticosteroids and antibiotics are often used in cases of mild ocular involvement, with one retrospective study suggesting that early topical steroids are associated with improved visual outcomes. Copious lubrication is essential for preserving ocular surface.
4. What is the role of early Amniotic membrane graft (AMG) in acute SJS?
Amniotic membrane is the innermost layer of the placenta. Its value in ocular surgery lies in its ability to enhance epithelisation, reduce inflammation, inhibit neovascularization, and prevent ocular surface scarring.
Various studies support the effectiveness of AMG in minimizing long-term visual sequelae of SJS. These studies emphasize the importance of early AMG intervention. Outcomes are patient-dependent, but results indicate that delay in treatment beyond 5 to 10 days after rash onset are associated with decreased visual acuity and increased ocular complications. The entire ocular surface from lid margin to lid margin should be draped, for maximizing the benefit of AMG. Patients undergoing AMG only to the bulbar conjunctiva may still develop the chronic sequelae of SJS.
5. What is Mucous Membrane Graft (MMG) and at what stage it is required in SJS?
In late stage of SJS, there may be development of keratinisation of lid margin, which causes rubbing of cornea (like a sand-paper) leading to corneal erosion and pannus formation.
In mucous membrane graft, oral mucosal membrane of the patient is harvested and is transplanted over the undersurface of eyelids, after removal of keratinized layer. MMG gives dramatic improvement to the patient in terms of eye opening and comfort.