Stevens-Johnson Syndrome

Image of Stevens-Johnson syndrome impacting the eye and amniotic membrane graft.

Stevens-Johnson syndrome is a rare condition that is often a reaction to a medication or an infection. This condition is an emergency.


Symptoms


Patients might first experience symptoms of the flu, such as a fever, sore mouth and throat, fatigue, cough, and burning eyes, and then a painful rash that spreads and blisters. Patients might have body aches, drooling due to difficulty closing the mouth, and eyes sealed shut by blisters and swelling.


The top layer of the affected skin necrotizes, sheds, then heals. It may take weeks or months to recover. You will have to discontinue any medication that is found to be the cause.


How does Stevens-Johnson Syndrome Affect the Eyes?


All mucus membranes are affected. 50% of patients have ocular symptoms. Stevens-Johnson syndrome can lead to inflammation of the eyes, and/or conjunctivitis which on the one hand might only result in irritation and dry eyes, but on the other hand can cause widespread tissue damage and scarring. Patients might also be affected by iritis, corneal blisters and perforation. Scarring will affect your eyesight, and in very rare cases, can cause blindness.


Hospitalization and Treatment
You might be treated in ICU or a burn unit.


Doctors will focus on treating the underlying cause of Stevens-Johnson syndrome, and controlling symptoms. The skin must regrow. The doctor will try to manage complications, replace electrolytes with IV fluids, use antibiotics to prevent infection, provide pain relief medications.


To treat the eyes, therapy can range from artificial tears in the case of inflammation and dry eyes to corneal transplants or limbal stem cell transplants.


Often, Stevens-Johnson syndrome symptoms of the eye are treated with amniotic membrane transplants. Like with conditions like symblepharon (which can be caused by Stevens-Johnson syndrome) AMT can result in rapid healing of the eyes. Early intervention is best.


Causes
There are several kinds of medications that can cause Stevens-Johnson syndrome, especially sulfa drugs, and including certain anti-gout medications, anticonvulsants and antipsychotics (especially if you are undergoing radiation therapy), medications to fight infection, and even pain relievers.


Herpes simplex or zoster, pneumonia, HIV, and Hepatitis A can all trigger Stevens-Johnson syndrome.


You might be at greater risk of Stevens-Johnson syndrome if you have a weakened immune system (including from an organ transplant), a history or family history of the syndrome, or a certain gene that is more common in families of Chinese, Southeast Asian, or Indian descent.


Other Risks
Risk factors include having a bone marrow transplant, lupus, or other chronic diseases of joints and connective tissue. More females than males get Stevens-Johnson syndrome, and it is more common in individuals under 30.


Complications
There are some complications you must consider if you suffer from Stevens-Johnson syndrome.


First, it is possible to get cellulitis, a secondary skin infection, or a blood infection (sepsis). The eye problems that can result are discussed above. Inflammation and other problems in the lungs can lead to trouble breathing, pneumonia, and respiratory failure.


The rash from Stevens-Johnson syndrome might lead to permanent skin damage, from abnormal texture and color, scarring, to hair loss and abnormal growth of finger and toenails.


Conclusion
Seek immediate emergency help if you have the symptoms of Stevens-Johnson syndrome. Treatments are available.