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Stevens-Johnson syndrome

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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.

Causes

- 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.

Symptoms

- 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.

Complications

- 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

- Diagnosis is usually confirmed by the appearance of lesions.

- A skin biopsy may be taken.

Treatment

- 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.

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