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Steven Johnson Syndrome… Not a skin problem alone!

Mrs. Sunita Kolhekar*,a 73 years old lady was in Singapore when she suddenly developed fever with rashes all over the body. She had developed this after taking some antidepressant medications and was diagnosed to have Steven Johnson Syndrome (explanation below). She was prescribed oral medications and skin ointments. Subsequently she flew back to India for personal reasons. In India, Mrs. Sunita started complaining of a sensation of having some object in both eyes with blurring of vision.

Sunita consulted Dr Pooja Jain, who is an Eye Specialist at Advanced Eye Hospital in Navi Mumbai. When Dr Jain examined her eyes, her best corrected vision (vision that is best possible even after optimum spectacle power) in both eyes was 6/18 (This means that she could see at 6 metres what a normal person can see at 18 metres).

Dr Pooja Jain examined Sunita’s eyes on the slit lamp microscope which showed that she had severe Meibomian Gland Dysfunction. Meibomian Glands are responsible for maintaining the lubrication of one’s eyes. Her cornea, the outer transparent layer of the eye, also had various dry spots. She had also begun developing Cataract in both her eyes, which was the main reason for her loss of vision. The outermost layer of her eye, the conjunctiva was congested.  Both her eyes showed that there was mild scar tissue (called fibrosis) below her conjunctiva. This had formed adhesions (bands of scar tissues joining two layers) between her eyelid and conjunctiva.    

She was diagnosed to have ophthalmic manifestations of Steven Johnson syndrome and started on hourly lubricating drops, bed time lubrication gel and steroid eye drops for the inflammation, which were tapered on a weekly basis. In addition she was prescribed cyclosporine eye drops. She was advised to do hot fomentation over her closed eye lid margins for 5 minutes at least twice a day and asked to follow up on a monthly basis.

Stevens-Johnson syndrome (SJS) is a rare, acute disease involving the skin and mucous membrane. It most commonly occurs as a reaction to medications or infections, and it produces a clinical picture similar to that of an extensive skin burns. Its effects on the eye surface can be devastating, leaving survivors with permanent, severe dry eye problems and debilitating sensitivity to light. In the mild form, there is discomfort and sensitivity to light. In the severe cases, there is destructive inflammation of the conjunctiva. The raw surfaces can lead to bands of scar tissue (adhesion) between the conjunctiva covering the inner surface of the eyelid and the eyeball, known as symblepharon. The normal mucosal structure of the conjunctiva is eventually replaced by scar tissue.

 

   

                                                                          Bands of Scar Tissue between Eyelid and Eye Surface

 

When this scar tissue contracts (as all scars tend to), there can be an outward turning of the eyelid (called entropion). Lid margin inflammation can cause widespread destruction of meibomian gland openings and the glands themselves. The structure of the eyelashes can also be affected, resulting in conditions like ingrowth of eyelashes. The abnormally directed lashes can rub against the eye surface and lead to discomfort, abrasions & ulcers on the cornea. Damage continues every time one blinks. This damages the cornea, and accompanied by the abnormal tear film (thanks to the abnormal meibomian gland), produces severe visual loss.

Take Home Message

  1. A patient diagnosed with SJS requires regular ophthalmic followup.
  2. Prevention of symblepharon is easier than surgical treatment.
  3. SJS patients require a lot of moral support as the disease has a long course with frustrating symptoms.

*Name Changed To Protect Privacy