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A5351 - Skin Sloughing and Hypoxic Respiratory Failure
Author Block: R. D. Rogers1, R. Young1, B. Dembo2; 1Pulmonary Critical Care, Penn State Milton S. Hershey Medical Center, Hershey, PA, United States, 2Internal Medicine, Penn State Milton S. Hershey Medical Center, Hershey, PA, United States.
Introduction: We report the first documented adult developing pneumomediastinum as a consequence of Stevens Johnson Syndrome. Case Description: A 25 year old male with Adult Still’s Disease in remission underwent a dental procedure a week prior to hospitalization and received clarithromycin and tramadol. Shortly thereafter he started to develop bilateral eye discomfort, odynophagia, and skin ulcers noted throughout his body. Dermatology was consulted and performed a punch biopsy of the lesions, which was consistent with Steven Johnson Syndrome (SJS). Workup was initiated for infectious causes of SJS and all came back negative; the cause was attributed to the aforementioned medications. He was placed on pulse dose steroids, and aggressive supportive care was utilized. Throughout the course of his hospitalization, he developed an increasing oxygen requirement. Chest x-ray showed a right lower lobe infiltrate, attributed to aspiration pneumonia, and he was started on antibiotics without improvement in his oxygenation. A CT scan of his chest was performed due to persistent hypoxemia and showed pneumomediastum and small right pneumothorax. Oxygen requirements continued to worsen, and the patient required mechanical ventilation for six days but eventually improved. He was subsequently extubated. Serial x-rays were performed and showed resolution of the pneumomediastum. His skin lesions began to heal, and he was discharged home. Discussion: Medications and infections are notorious for causing the mucocutaneous involvement of SJS. SJS has an estimated prevalence of 1-2 case per million inhabitants per year develop the syndrome and is fatal in 10-30% of the cases. Leading causative agents include NSAIDs, antimicrobials and anticonvulsants. Our focus in this case report is not the known side effects of clarithromycin and tramadol but rather the rare complication of the condition itself. Pneumomediastum is commonly associated with surgical procedures, respiratory maneuvers and certain medical conditions such as infections, asthma and cystic fibrosis; it is rarely seen with SJS. Two publications have reported pneumomediastinum in children ages 9 and 10 with SJS, but this complication has never been reported with adults. The mechanism of action is postulated to be from direct pulmonary parenchymal involvement in the proximal or distal airways causing micro-perforations and allowing air to enter the mediastinum. SJS is associated with such pulmonary complications as pneumonia, pneumonitis and bronchiolitis obliterans; although extremely rare, pneumomediastum and pneumothorax should also be considered. To the best of our knowledge, this is the first reported case of an adult developing pneumomediastum as consequence of Stevens-Johnson Syndrome.