Disclosures: Maya Tarasenko, BS: No financial relationships or conflicts of interest

Case Diagnosis: Quadruple limb amputation following Stevens Johnson Toxic Epidermal Necrolysis Overlap Syndrome (SJS/TENOS) secondary to treated bowel perforation and intra-abdominal abscess.

Case Description or Program Description: A 40-year-old female with chronic low back pain, long-term naproxen and cocaine use presented with 2 days of coffee ground emesis and worsening abdominal pain. She was found to have a gastric perforation complicated by acute renal failure, fungemia, bacteremia, intraabdominal abscess, and enterocutaneous fistula. She developed SJS/TENOS, possibly resulting from antibiotic therapy, requiring quadruple limb amputation.

Setting: Inpatient Rehabilitation Hospital

Assessment/Results: The patient completed a 3-month acute rehabilitation hospitalization. Her recovery was complicated by phantom limb pain, a stage 2 sacral decubitus ulcer, and large open lower extremity wounds bilaterally. Ultimately, she made exceptional progress in self-care tasks and prostheses management. While the patient continued to demonstrate impairments in range of motion, endurance, and overall strength and coordination, she was ultimately discharged home with 24/7 assistance and outpatient therapy.

Discussion (relevance): The annual incidence of SJS is 1.2 to 6 cases per 1,00,000; the incidence of TEN is far less. Most commonly precipitated by medications in the setting of genetic predisposition and immunosuppression, SJS/TENOS typically presents with prodromal fever followed by cutaneous manifestations. Anemia, ocular disturbances, pneumonitis, dysphagia, and acute kidney injury are common complications. Limb amputations, however, are not. In fact, SJS/TENOS typically begins on the trunk, spreading to the neck and face. Distal extremities are often spared. To the best of our knowledge, this is the first recorded case of a quadruple limb amputation secondary to SJS/TENOS and brings awareness to the extent and severity of associated complications.

Conclusions: SJS/TEN is known to cause serious and lasting effects, however, necrolysis requiring quadruple limb amputation demonstrates a new extent of morbidity.

Level of Evidence: Level V

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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/rehabilitation-of-quadruple-limb-amputation-following-stevens-johnson-toxic-epidermal-necrolysis-overlap-syndrome-a-case-report/