Abstract
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.
Keywords
conjunctiva - cornea - lids
