Management of Stevens-Johnson syndrome

A patient with Stevens-Johnson syndrome is described, and the literature concerning the etiology, pathophysiology, clinical manifestations, and management of Stevens-Johnson syndrome is reviewed. A 2 1/2-year-old girl was treated with phenobarbital and i.v. ampicillin, followed by oral amoxicillin, for an upper-airway infection, otitis media, and febrile seizures. The fever returned, and she was treated unsuccessfully with penicillin and cefaclor. She was admitted to the hospital and treated with i.v. ampicillin. Within 24 hours an erythematous maculopapular rash developed. Phenobarbital was discontinued and phenytoin was begun. Four days later bullous lesions developed; ampicillin and phenytoin were discontinued, and cefazolin and phenobarbital were given. By the eighth day severe sloughing of the skin occurred over 75% of her body, and mucosal sloughing was apparent. The patient's condition was diagnosed as Stevens-Johnson syndrome. Porcine xenografts were immediately grafted to 75% of her total body surface. Severe lesions of the mouth and pharynx made parenteral nutrient therapy necessary, and ocular complications required the care of an ophthalmologist. Although the skin had healed by 14 days after grafting, another 14 days of treatment for respiratory complications was required. Stevens-Johnson syndrome is a severe exfoliative dermatitis accompanied by fever, inflammation of the gastrointestinal mucosa, and severe purulent conjunctivitis. It is associated with high morbidity and mortality. The etiologic factors may be iatrogenic (e.g., various antibiotics and anticonvulsants), infectious, or idiopathic. Respiratory complications, leukopenia, infections, erosion of the gastrointestinal mucosa, fluid and electrolyte disturbances, and chronic ocular complications may occur.(ABSTRACT TRUNCATED AT 250 WORDS)