All you need to know about Stevens-Johnson Syndrome

Stevens-Johnson syndrome is a severe form of erythema multiforme, associated with a mortality rate of 10%. Complications include blindness, renal failure, meningitis, necrotizing tracheobronchitis, dehydration, secondary bacterial infection, arrhythmias, and congestive heart failure.

Who is commonly affected?

Children and young adults are most frequently affected by the disease. 

CLINICAL FEATURES

1. Upper respiratory tract infection, headache, fever, hematuria, diarrhea, and arthralgias can precede the rash.

2. Rash: Skin lesions are burning but not itchy

a) Bullae appear in 1 to 14 days on the skin and the mucous membranes of the mouth, genitalia, and anus.

b) Ulcers: Corneal ulcers can lead to blindness. Ulcerative stomatitis leads to hemorrhagic crusting. Patients are unable to eat or drink and continuously drool.

c) Vesicles rupture and leave denuded bases and necrotic epithelium.

3. Signs of toxic epidermal necrolysis (TEN) may develop. Urinary retention can result from urethral involvement.

DIFFERENTIAL DIAGNOSES include TEN and pemphigus.

EVALUATION: Skin biopsy findings include necrolysis with edema and erythrocytes in the dermis.

THERAPY

1. Definitive treatment entails treating the cause if it can be identified (e.g., with antibiotics or termination of drug therapy). Prednisone (80 to 120 mg/day in divided doses) can be administered orally with subsequent tapering. Intravenous immunoglobulin has also been used successfully to halt the progression of TEN. It is important to note that treatment with prednisone or intravenous immunoglobulin is controversial and varies from one institution to another.

2. Supportive treatment

a) Cool, wet compresses

— Aluminum acetate compresses are applied to blisters.

— Compresses soaked in potassium permanganate solution are applied to bullous lesions.

b) Anesthetic troches, 2% viscous lidocaine, or 10% sodium bicarbonate mouthwashes can be used to soothe mouth lesions. If the patient cannot tolerate a liquid diet, he or she will require intravenous rehydration.

c) Antibiotic therapy is indicated for patients with secondary bacterial infections.

d) Ophthalmology consult: An ophthalmologist should be consulted.

d) Urology consult: Urology should be consulted if genitourinary involvement is suspected.

DISPOSITION: Patients with severe mucous membrane involvement require admission to a burn unit for reverse isolation and treatment of fluid and electrolyte imbalances.