Stevens-Johnson syndrome

What is Stevens-Johnson syndrome?

Stevens-Johnson syndrome (SJS) is a serious skin condition that arises as a reaction to a medication or an infection. It is sometimes called erythema multiforme major. It is much less common than erythema multiforme minor.

SJS tends to affect children and young adults. The condition often starts with a minor upper respiratory infection and a few days later the patient suddenly becomes very unwell. The main features of SJS are:

• Red blistered, eroded, bloody or crusted lips (cheilitis), mouth (stomatitis) and genitals (mucosal ulceration)
• Red, sticky and painful eyes (conjunctivitis), which may become scarred
• Target lesions on hands and feet (and sometimes elsewhere) in some patients
• Small to large red tender patches that blister and may peel off but do not involve more than 10% of body surface in some patients
• High fever and systemic toxicity
• Swollen lymph glands
• Some patients may develop pneumonia or involvement of other organs including joints, liver, heart, kidneys and gastrointestinal tract.

The features of SJS may be indistinguishable or overlap with the related skin condition toxic epidermal necrolysis (TEN). However, most dermatologists diagnose TEN if more than 30% of body surface area is affected by denuding skin. They diagnose SJS/TEN overlap syndrome if between 10 and 30% of body surface area is affected.

Stevens-Johnson syndrome

What is the cause of SJS?

SJS is often caused by medications taken 2 weeks to 2 months prior to the onset of the reaction. These include:

• antibiotics containing penicillin, sulfa or tetracyclines
• nonsteroidal anti-inflammatory drugs including naproxen and ibuprofen
• anticonvulsants such as carbamazepine or phenytoin
• allopurinol

It may also follow Mycoplasma pneumonia (a chest infection), and is sometimes seen in epidemics due to this cause. Other bacterial or viral infections are less commonly responsible.

Complications of SJS

During the acute illness, complications of SJS may include:

• dehydration
• bacterial infection of the skin, mucosa or lungs

Although many patients with SJS have a short lasting illness and recover fully, SJS may result in significant long term skin and mucosal problems. These include:

• Scarring and pigment change of the skin and mucosal surfaces
• Loss of nails and permanent scarring (pterygium) preventing regrowth
• Joint contractures
• Serious eye problems including corneal scarring and blindness

Management of SJS

SJS requires admission to hospital for observation, intravenous fluids and nursing care. Severe cases are cared for in an intensive care facility or burns unit. It may take weeks to months for symptoms and signs to settle.

Sometimes a skin biopsy is necessary to confirm the diagnosis.

Treatment may include:

• Discontinuing any medication that could be responsible
• Correction of fluid and electrolyte imbalance and nutritional deficits
• Skilled nursing care, pain killers, physiotherapy and wound dressings
• Antibiotics for secondary infection

The use of other agents is unclear but includes Ciclosporin and Intravenous immunoglobulin.

Systemic corticosteroids may aggravate the condition