Stevens‐Johnson Syndrome

Stevens-Johnson Syndrome

Susan Haller Psaila, MD

Basic Information

Definition

Stevens-Johnson syndrome (SJS) is an exfoliative dermatitis with severe erosions of at least two mucosal surfaces, including extensive necrosis of oral and nasal mucosa and purulent conjunctivitis but less commonly involving vaginal, urethral, gastrointestinal, or respiratory mucous membranes.

Synonym

Erythema multiforme major

ICD-9-CM Codes

695.1 Erythema multiforme major, Stevens-Johnson syndrome

Epidemiology & Demographics

• The exact incidence is unknown.
• Peak incidence is in the second decade of life.
• SJS is more common in the spring and summer.

Clinical Presentation

• Between 1 and 14 days after a prodrome of malaise with fever or other flulike symptoms, there is an abrupt onset of symmetric, red macules that progress to central blistering and potentially extensive epidermal necrosis.
• The extent of skin involvement varies.
• Lips develop hemorrhagic crusts, with loss of the mucosa and severe stomatitis.
• Purulent conjunctivitis with photophobia and pseudomembrane formation may develop.
• Anogenital mucosa also may be involved.
• Esophageal, respiratory, and nasal mucosa are occasionally involved.
• Generalized lymphadenopathy and hepatosplenomegaly are usually present.
• Signs of dehydration (e.g., tachycardia, hypotension) may be observed.

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Jul 3, 2016 | Posted by in PEDIATRICS | Comments Off on Stevens‐Johnson Syndrome

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