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Case 2

An 18-year-old male patient presented to the emergency department complaining of four days of increasing dysphagia, dysuria, photophobia, and a macular rash extending from the trunk toward the

extremities. The only medication used by the patient was tetracycline, which he had been taking for two weeks as treatment for facial acne. Vital signs were normal except for a temperature of 103.1°F. He

appeared ill and had copious amounts of ocular drainage as well as small vesicles on the nasal and oral mucosa. An erythematous rash on his chest coalesced on the trunk with many small vesicles, some

forming bullae. Vesicles were also present on the penis and scrotum.

On investigation, the patient's white blood cell count was slightly elevated at 11.7 × 109/L. Blood, herpes, and mycoplasma cultures as well as results of both rapid plasma reagin test and anti-DNA test

were negative; and results of a skin biopsy were consistent with Stevens-Johnson syndrome. The presumptive cause was tetracycline.

Empirical therapy with acyclovir was started but was discontinued after results of herpes culture proved negative. A regimen of 60 mg prednisone given intravenously twice daily was also started. When

the oral lesions became so painful that the patient could not swallow his own saliva, a regimen of total parenteral nutrition was started, and the patient was given a patient-controlled anesthesia pump for

administration of morphine. As the vesicles spread, they coalesced into larger bullae and sloughed off. The skin lesions were treated twice daily with a mixture of urea and triamcinolone in a lotion base.

Multiple chest x-ray films showed no pulmonary involvement. Because of his need for increasing wound care, the patient was transferred to the intensive care unit. Ophthalmologic and urologic

consultation was obtained to address ocular and urethral symptoms.

The area of denuded skin increased, and this development required even more labor- intensive treatment; the patient was therefore transferred to the county burn unit for wound management. His

condition improved during the next two weeks, and he eventually recovered with minimal scarring on the back. Follow up continued an outpatient basis in the ophthalmology, dermatology, and urology

departments.

Please discuss, in depth, each of the following related to Stevens-Johnson syndrome using evidence-based resources:

1. Incidence

2. Course of the disease

3. Etiology

4. Treatment

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