Blindness in patients with a devastating disease called Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) can be prevented by transplanting tissue from newborn fetal membranes. Unlike before, SJS/TEN are now believed to be variants of the same condition, distinct from erythema multiforme. SJS/TEN is a very rare, acute, serious, and potentially fatal skin reaction in which there is sheet-like skin and mucosal loss. Using current definitions, it is usually, possibly always, caused by medications. Anyone on medication can develop SJS/TEN unpredictably. It can affect all age groups, both sexes and all races. It is more common in association with human immunodeficiency virus infection (HIV), which may reflect the increased use of medications by HIV patients.
Before the rash appears, there is usually a prodromal illness of several days duration resembling an upper respiratory tract infection or ‘flu-like illness. Symptoms may include fever – persistent and high, cough, sore throat, difficulty swallowing, runny nose, sore red eyes, conjunctivitis and general aches and pains. There is then an abrupt onset of a tender/painful red skin rash starting on the trunk and extending rapidly over hours to days onto the face and limbs. The maximum extent is usually reached by 4 days. The skin lesions may be macules, targets and blisters. In addition to skin/mucosal involvement, other organs may be affected including liver, kidneys, lungs, bone marrow and joints. SJS/TEN has very rarely been associated with vaccination. The most commonly implicated medications are antibacterial sulfonamides.
A new eye treatment for SJS/TEN patients is called amniotic membrane transplantation. Amniotic membrane is part of the fetal membranes that surround and protect the baby in the womb, and have natural therapeutic properties. When placed on the eye, amniotic membrane can help aid healing, decrease inflammation and minimize scarring. Amniotic membrane is donated by a consenting mother following the birth of her baby. SJS/TEN has an earlier acute stage and a later chronic stage. Amniotic membrane transplantation is effective in the chronic stage. The use of early amniotic membrane transplantation, within the first three to five days, over the entire ocular surface is supported. If the amniotic membrane is placed more than one week after the onset of the disease, the beneficial effects may be reduced. The levels of a variety of inflammatory mediators in the skin, blood, eye and mouth in patients with SJS/TENS to better understand the cause of this devastating disease, and to develop better treatments is the topic of research now.