Stevens Johnson Syndrome/Toxic Epidermal Necrolysis - Diagnostic Testing and Treatment for Ophthalmologists

Epidermal necrolysis (EN) is a very rare, but life-threatening dermatological disease where the skin and various mucous membranes react to drugs (75%) or systemic infections (25%). If up to 10% of the body surface is affected, the disease is called Stevens Johnson syndrome (SJS); if more than 30% of the body surface is affected, it is called toxic epidermal necrolysis (TEN); if 10 - 30% of the body surface is affected, it is called SJS/TEN overlap. In the acute phase of the disease, 80% of cases show ophthalmic involvement (from mild conjunctivitis to total de-epithelialisation of the ocular surface). Therefore, patients should be seen by an ophthalmologist within 24 h after symptoms have started. Treatment of the acute ophthalmic problems has a very important impact on long-term eye complications. However, in the long run up to 50% of patients develop chronic sight threatening ocular surface disease which needs further intensive topical and surgical treatment (e.g. lubrification, topical anti-inflammatory agents, lid surgery, scleral lenses, corneal surgery). The indication for corneal transplantation (allogenic penetrating limbo-keratoplasty and keratoprostheses) should be given with due caution, as the prognosis is very poor.