Photo Rounds

Rash with fever and shortness of breath

J Fam Pract. 2019 July;68(6)

A 35-year-old HIV-positive man presented to the emergency department with fever, shortness of breath, and a rash on his face. His chest X-ray suggested pneumonia, so he was admitted to the hospital and started on azithromycin, ceftriaxone, and trimethoprim/sulfamethoxazole. His rash worsened rapidly over the next 2 days (affecting > 30% of his skin) and he developed oral lesions. A dermatologist was called to see him when he developed bullae. The dermatologist performed a shave biopsy under one of the smaller bullae and sent the specimen to the pathologist, stat.

What’s your diagnosis?

 

Rash with fever and shortness of breath

The skin biopsy confirmed toxic epidermal necrolysis (TEN), possibly secondary to one of the antibiotics he’d been given upon hospital admission. The health care team suspected that the patient had Stevens-Johns Syndrome when he sought care and that it had evolved into TEN. Both diseases are life-threatening and require intensive in-hospital care.

TEN is part of a spectrum of disorders that includes erythema multiforme (< 10% of the body surface is involved); SJS/TEN (10%-30% involvement with erythematous or pruritic macules, widespread blisters on the trunk and face, and erosions of ≥ 1 mucous membranes); and TEN (> 30% involvement).

Drugs most commonly known to cause SJS and TEN include sulfonamide antibiotics, allopurinol, nonsteroidal anti-inflammatory agents, amine antiepileptic drugs (phenytoin and carbamazepine), and lamotrigine. Fifty percent of SJS/TEN cases have no identifiable cause. Not all SJS is secondary to drug exposure, but it is the job of the clinician to investigate this cause and stop any suspicious medications.

In this case, trimethoprim/sulfamethoxazole was discontinued immediately and the patient was transferred to a burn unit and given intravenous gamma-globulin 1 g/kg for 3 days. Fortunately, the patient survived with intensive supportive care in the burn unit. The exfoliation of > 30% of the skin is like a large secondary burn, and a burn unit is the optimal location for lifesaving measures.

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Photo courtesy of Robert T. Gilson, MD, and text for Photo Rounds Friday courtesy of Richard P. Usatine, MD. This case was adapted from: Milana C, Smith M. Erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis In: Usatine R, Smith M, Mayeaux EJ, et al. Color Atlas and Synopsis of Family Medicine. 3rd ed. New York, NY: McGraw-Hill; 2019:1161-1168.

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