DESCRIPTION
Severe blistering mucocutaneous syndrome involving at least two mucous membranes.

HISTORY

• Occurs in all ages but more common in children and young adults.
• Thought to be due to cytotoxic immune responses directed against keratinocytes expressing foreign infectious {Mycoplasma pneumoniae) and drug antigens (phenytoin, phenobarbital, carbamazepine, sulfonamides, amino-penicillins).
• Medications started within 1 month of disease onset are more likely to cause Stevens-Johnson syndrome.
• HIV infection, systemic lupus erythematosus, and malignancies treated with radiation increase the risk.
• Can involve pulmonary, gastrointestinal, renal, and central nervous systems.

PHYSICAL FINDINGS

• Erythematous papules, dusky-appearing vesicles, purpura, and target lesions erupt acutely. Lesions can be tender and burn.
• Oral, genital, and perianal mucosa develop bullae and erosions.
• Thick hemorrhagic crusts can cover the lips.
• Patients develop conjunctivitis and are at risk for corneal ulceration and uveitis.
• Stevens-Johnson syn­drome skin lesions are more centrally distributed on the face and trunk.
• Crops of lesions erupt for 10-14 days and slowly subside for the next 3-4 weeks.

TREATMENT

• Identify and treat sources of infection, withdraw suspected offending drugs, maintain fluid and nutritional requirements, provide meticulous local wound care.
• Mucous membranes: frequent mouth rinses and applications of petroleum jelly (Vaseline) or Aquaphor.
• Viscous lidocaine (Xylocaine) or Benadryl elixir for pain.
• Topical erythromycin ointment to the eyes prevents ocular adhesions.
• Treat eroded skin like a burn: cleanse gently, remove necrotic tissue, apply bland emollients.
• Narcotics for severe pain.
• Intravenous immunoglobulins in selected patients.
• The use of systemic corticosteroids is controversial. Cortico­steroids may be beneficial in certain cases. Sick children who have extensive cutaneous, ocular and oral lesions may respond to prednisone (20 to 30 mg twice a day) until new lesions no longer appear; it is then tapered rapidly.