Stevens-Johnson Syndrome: A Potentially Deadly Skin Disease

So, what really causes Stevens-Johnson syndrome?

Symptoms of SJS

Be on the alert when severe skin rashes appear after taking certain drugs.

In January 2012, a polytechnic student allegedly passed away because of an allergic reaction to acne medication.

The same month, a 16-year-old youth with a pre-existing epilepsy condition experienced blurry vision and a severe rash after being treated with carbamazepine, an anti-convulsant drug.

Both are believed to have developed Stevens-Johnson syndrome, also known as SJS or erythema multiforme.

Dr Pang Shiu Ming, Senior Consultant from the Department of Dermatology at Singapore General Hospital (SGH), a member of the SingHealth group, explains, "This is a rare and potentially life-threatening skin disease characterised by a red, painful and blistering rash all over the body."

So, what really causes Stevens-Johnson syndrome?

The most common cause of SJS is a severe reaction to certain medications. To a lesser extent, Stevens-Johnson syndrome has been linked to viral or bacterial infections. However, in some cases, doctors are not able to determine the exact cause of the disease.

The medications which have a link to SJS include:

Anti-convulsants like carbamazepine, phenytoin, phenobarbital and lamotrigene
Anti-gout medications like allopurinol
Antibacterial sulfonamides like dapsone and trimethoprim-sulfamethoxazole
Oxicam-nonsteroid anti-inflammatory drugs like piroxicam and meloxicam
Anti-HIV drugs like niverapine

Infections include:

Herpes simplex
Mycoplasma

Drugs are more implicated in adults, and infections are more implicated in children.

Genes can also play a part. "Carriers of the human leucocyte antigen HLA-B1502 in Han Chinese are associated with carbamezapine-induced SJS, and HLA-B5801 associated with allopurinol- induced SJS," says Dr Pang.

Symptoms of SJS

Often, Stevens-Johnson syndrome begins with non-specific symptoms such as fever, sore throat and irritation in the eyes. These flu-like symptoms are often misdiagnosed by doctors and treated with antibiotics.

As the condition worsens, more specific symptoms develop:

Skin lesions:
- Red or purplish patchy rash that may appear on the face, arms and legs. It may be symmetrical on both sides of the body, and may appear to have a central sore encircled by pale red rings (known as a “bulls-eye”). This rash may erupt as painful blisters.
- Ulcers and other lesions in mucous membranes like the mouth, eyes and genital regions
Eyes may be dry, bloodshot and irritated in mild cases. Or, they may burn and itch, with a discharge in severe cases. Such inflammatory symptoms may lead to vision problems such as blurry vision and eventually, blindness.

In drug-related cases, it usually takes one to two weeks or a few weeks (from the time the patient first consumes the medications) for these symptoms to manifest.

Read on for treatment details on Stevens-Johnson syndrome.

Ref: T12

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