VOL: 102, ISSUE: 17, PAGE NO: 21
What is it?
- Stevens-Johnson syndrome is an inflammatory condition that affects children and young adults. It can be fatal.
- It is classified as an immune-complex-mediated hypersensitivity condition and is a variant of bullous erythema multiforme.
- It can range from a mild skin condition to a severe systemic disorder.
- The most common cause in adults is allergic reaction to drugs. More rarely it can be caused by a malignancy.
- Infections are the most common cause in children, including viral infections such as mumps, bacterial infections including diphtheria, fungal infections such as dermatophytosis and protozoal infections such as malaria.
- Immunisation associated with measles, for example, can cause the syndrome in children.
- Up to half of cases have no known cause.
- Stevens-Johnson syndrome affects the mucous membranes of the mouth, nostrils, eyes, and anal and genital areas.
- It usually begins with a non-specific infection of the upper respiratory tract including sore throat, fever, chills, headache and vomiting.
- Mucocutaneous lesions develop in clusters. These can occur anywhere on the body, but most commonly appear on the palms, dorsum of hands, soles and extensor surfaces.
- The lesions can last for 2-4 weeks but are not pruritic. Painful oropharyngeal lesions may make eating difficult. The lesions may be target lesions or bullae.
- Severe conjunctivitis often occurs and can lead to corneal scarring and impaired vision.
- Genital involvement can lead to dysuria or inability to void.
- Fever and fatigue are common.
- Approximately one-third of patients have pulmonary involvement, which results in coughing. X-ray will reveal patchy infiltrates.
- In severe cases renal failure and pneumonia result in death.
- Corneal ulceration, anterior uveitis, panophthalmitis, blindness.
- Oesophageal strictures.
- Renal tubular necrosis, renal failure.
- Penile scarring, vaginal stenosis.
- Tracheobronchial shedding with respiratory failure.
- Scarring and cosmetic deformity.
- Diagnosis is usually confirmed by the appearance of lesions.
- A skin biopsy may be taken.
- Treatment of Stevens-Johnson syndrome is mainly supportive. It may include the following:
- Monitoring of airway and haemodynamic stability;
- Replacement of electrolytes and fluid;
- Management of the underlying cause, including discontinuing any complicating medication;
- Pain control;
- Mouthwashes and topical anaesthetics to reduce pain and allow eating and drinking;
- Immunoglobin therapy;
- Antibiotics to control secondary skin infections;
- Steroids to reduce the risk of severe infection, although their use has been associated with increased complications.
- Skin lesions should be treated as burns.