Clinical Manifestations

Most patients with SJS have a distinct prodrome of an upper respiratory illness with fever, sore throat, rhinitis, malaise, vomiting, and diarrhea 1 to 14 days prior to the mucocutaneous eruption. Two or more mucosal sites must be involved to make the diagnosis of SJS. The oral mucosa is always involved with extensive superficial necrosis of the lips and mouth, leading to hemorrhagic crusts and followed by denudation of the mucosa and severe stomatitis (Fig. 7). Purulent conjunctivitis with photophobia can be seen. Anal and genital mucosa, and less commonly the esophagus, respiratory epithelium, and nasal mucosa can also be involved.

Most patients with SJS have skin lesions in addition to the mucosal involvement. The skin lesions usually start as red macules and may be limited to a few targetoid lesions or may evolve rapidly into widespread dusky-red macules, which can become confluent.

FIGURE 7 Extensive mucosal necrosis of the lips (A) and severe stomatitis (B) in a patient with Stevens-Johnson syndrome. Courtesy of Dr.

FIGURE 7 Extensive mucosal necrosis of the lips (A) and severe stomatitis (B) in a patient with Stevens-Johnson syndrome. Courtesy of Dr.

The lesions are usually tender and the patient is usually unable to eat or drink. Patients with SJS appear acutely ill and may have generalized lymphadenopathy or even hepatosple-nomegaly. Arthralgias, hepatitis, nephritis, myocarditis, and gastrointestinal bleeding are occasionally seen.

An elevated erythrocyte sedimentation rate (ESR) is found in all patients with SJS; leukocytosis can be seen in more than one-half of patients. Other laboratory abnormalities include eosinophilia, elevated liver enzymes, leukopenia, proteinuria, and microscopic hematuria.

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