Stevens-Johnson syndrome: Twenty-three cases and their otolaryngologic significance

Stevens-Johnson syndrome is a symptom complex characterized by mucosal, ocular, and cutaneous lesions. Prodromal symptoms of upper respiratory infection or pharyngitis are most common. These are followed by target lesions of skin, sloughing of musous membranes, and ocular inflammation. Twenty-three cases are presented. Most patients (61 percent) were between 10 and 39 years of age. Relapses occurred in three patients. Five patients developed pneumonia, and there were two fatalities. Over one-half of the patients had serious ocular complications. The etiology of this syndrome is uncertain. Multiple allergic factors, drugs (especially long-acting sulfonamides), and infectious agents (most recently mycoplasma pneumoniae) have been implicated. This study does not support any single cause. The use of systemic steroids is generally accepted by most authors. The death rate in this study (10 percent) is essentially unchanged from early reports on patients without steroid therapy. The complications rate directly attributable to steroids is significant in this study group. The major complications of this syndrome are ocular. Aggressive topical management of ocular inflammation is required; however, the morbidity rate remains high despite these measures. The otolaryngologist should be aware of this syndrome as the early symptoms, and the acute complications may require his awareness of the natural and treated courses of this disease.