Managing Acute Stevens-Johnson Syndrome

In a Saturday afternoon session of the Cornea Subspecialty Day, Anthony Johnson, MD, emphasized the importance of early intervention in managing the ocular surface complications of Stevens-Johnson syndrome (SJS). This serious vesiculobullous disease of the skin can be triggered by 200 different medications, including such common drugs as sulfa antibiotics and ibuprofen, and has a mortality rate as high as 35% in its most severe form, known as toxic epidermal necrolysis (TEN). Although ocular involvement occurs in 50% or more of SJS patients, Dr. Johnson said that he has not observed much correlation between the severity of the skin and eye manifestations.

Systemic management. Because severe SJS/TEN causes sloughing of the epidermis, with resultant massive fluid loss, lack of protection against infection, and inability to control body temperature, these patients are generally managed in hospital burn centers. In a number of small studies, several forms of systemic treatment have been shown to reduce mortality—steroids, IV immunoglobulin, plasmapheresis, and TNF inhibitors—but their effect on eye disease is unclear.

Timely eye treatment a must. Because SJS patients are seriously ill and are being managed by burn specialists, their ocular problems may be overlooked until their eyes have been severely damaged by cicatricial changes and limbal stem cell loss. Dr. Johnson stressed the importance of assessing SJS patients as soon as possible for signs of ocular disease.

Any fluorescein staining of the conjunctiva, cornea, or fornix, or eyelid margin changes indicate the need for ophthalmic treatment. Dr. Johnson compared treatment to “cordoning off the fire” and emphasized that the goal is to deal with the inflammation primarily, rather than trying to catch up after the formation of symblephera and onset of cicatricial changes.

The key tool: amniotic membrane. The mainstay in treatment of SJS/TEN eye disease is amniotic membrane (AM) transplantation, pioneered by Dr. Shaeffer Tseng: It provides both anti-inflammatory effects and mechanical protection for the ocular surface and can be used in conjunction with steroids.

At his military hospital, Dr. Johnson said, “We line the entire ocular surface with amniotic membrane.” The goals are to 1) protect the meibomian glands, 2) preserve the fornix, and 3) prevent epithelial apposition and thus keep symblephera from forming. He and his colleagues follow this procedure:

• Trim the eyelashes to allow the AM to lie directly on the meibomian glands.
• Use IV tubing to form a “customized symblephon ring,” ensuring that the treatment goes from fornix to fornix.
• Make sure that the AM covers the entire ocular surface, including both the outside and inside of the eyelids, as well as the cornea and conjunctiva.
• Once the AM is laid down, suture it to the eyelid.

Dr. Johnson said that he performs most of these procedures at the patient’s bedside, though other surgeons may prefer to do so in an OR.

Lessons learned. Dr. Johnson offered the following key points to avoid severe or even blinding complications:

• Early intervention is crucial.
• Educate your burn staff to contact an ophthalmologist right away when an SJS patient presents.
• Always keep AM in stock.
• Watch the eyelid margins.
• Take care of the fornix.

—Peggy Denny

Dr. Johnson has no financial disclosures.