Stevens-Johnson syndrome and the eyes: ocular involvement and care

The British Association of Dermatologists (BAD) describes SJS and toxic epidermal necrolysis (TEN) as the milder and severe forms of the same condition: a rare, severe skin reaction characterised by a blistering rash in combination with inflammation of the eyes, mouth, nose and genitalia. The upper layer of skin can become detached, exposing the lower layer. In SJS the blistering involves less than 10% of the body surface area, while in TEN it involves more than 30%.

The NHS says SJS is often caused by the body reacting to certain medicines. Medicines commonly linked include allopurinol, carbamazepine, lamotrigine, nevirapine, oxicam anti-inflammatories, phenobarbital, phenytoin, sulfa antibiotics and sulfasalazine. It says SJS is rare and the risk of getting it is low even when taking a medicine known to cause it. In children it can also follow infections such as colds, flu, cold sores and glandular fever. BAD notes that almost any medicine can cause it, including over-the-counter and herbal preparations, and that sometimes no trigger is found.

BAD lists red, sore eyes among the earliest features, together with flu-like symptoms such as sore throat and fever, followed within days by a painful red or purple rash with inflammation of the mouth, lips, eyes and genitalia. It says involvement of the eyes can cause redness, weeping and crusting of the eyelids.

The NHS says blisters and sores can appear on the eyes, causing eye pain, pain when looking at bright lights and problems with sight. It also lists swollen or sore mouth, face, lips, tongue, throat or eyes among the signs that need emergency help.

The NHS says to call 999 or go to A&E if you or your child has a rash spreading across the body, or a circular rash that is darker in the middle and lighter around the outside, after an infection or starting a new medicine; has a rash with skin that is itchy or painful, red, swollen, blistered or peeling; is wheezing; has tightness in the chest or throat; has trouble breathing or talking; or has a swollen or sore mouth, face, lips, tongue, throat or eyes.

The NHS adds that you should not drive to A&E: ask someone to drive you or call 999, and take any medicines you are on with you. These can be signs of a serious reaction like SJS and may need immediate treatment in hospital.

BAD says there are no specific blood tests for SJS/TEN. A definitive diagnosis is made from the doctors' assessment of the skin, usually along with a skin biopsy, in which a very small sample of skin is examined under a microscope.

BAD says early treatment is important. If a drug is suspected as the cause it must be stopped immediately. You are admitted to hospital and, if skin loss is extensive, taken to an intensive care unit or burns unit. Treatment is supportive: minimising further skin loss, preventing infection, providing hydration and nutrition and pain relief, so that the skin can heal as quickly as possible once the reaction has finished. Topical treatments to the skin, eyes and mouth, consisting of ointments and, where necessary, dressings, are given. BAD says there is no clear evidence to show the benefit of any treatment with tablets or injections.

The NHS lists fluids into a vein, specialised wound care with creams and dressings, strong painkillers and medicines to control inflammation and prevent infection. It says without treatment symptoms can become life threatening. BAD describes a multidisciplinary approach that may include dermatology, ophthalmology, intensive care, burns, plastic surgery, respiratory and renal medicine, dietitians and physiotherapists, with intensive skin and mouth care from specialist nurses.

The NHS says it may take several weeks or months to recover fully, you may feel very tired for a few weeks after leaving hospital, and skin usually heals in 2 or 3 weeks. BAD says the skin, mouth, eyes and other areas may continue to be bothersome after discharge, and advises following the discharge instructions on moisturisers, eye care and mouth care and keeping all appointments.

The NHS lists eye problems, such as long-term inflammation or sight problems, among the complications of SJS, along with skin scarring and changes in colour, problems with the lungs, liver and kidneys, and scarring affecting the genitals. BAD says the skin will remain sensitive to sunlight for many months and recommends covering the skin, using at least SPF 30 sunscreen and wearing sunglasses outdoors. Ask your discharge team who is responsible for follow-up of your eyes and what eye care you should carry out at home.

The NHS says that if your symptoms were caused by a reaction to a medicine you will need to avoid taking that medicine, and possibly similar ones, for the rest of your life. BAD advises keeping a record of the offending drug, informing all medical practitioners, wearing a medical alert bracelet or pendant, not introducing any new medicine without discussing it with your doctor, including over-the-counter preparations, and avoiding herbal or natural preparations. In children where infection was the trigger BAD says there is a small chance it can recur, so medical attention should be sought promptly.

BAD describes SJS as treated in hospital, with intensive care or burns unit input when skin loss is extensive, and the NHS says it is treated in hospital, sometimes in an intensive care unit. Emergency care in the UK is delivered through NHS hospitals. After discharge, follow-up for eye and other complications is arranged by the hospital team, and a GP can help keep an accurate record of the medicine that triggered the reaction.