(648) Causative or Contributory? Interesting Presentation of TEN With Late Sjogren Diagnosis

Introduction: Patients presenting with skin findings consistent with Steven’s Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) often have unclear triggers for their skin condition, making the skin condition that much more challenging to treat and the prolonged time with non-intact skin increases the risk of complications, similar to burn patients with comparative TBSA. Many auto-immune disorders can trigger or increase likelihood of SJS or TEN, however, identifying these conditions in a critically ill patient can be challenging.

Description: A 64-year-old Hispanic female was admitted to our Burn unit with skin biopsy and clinical exam findings consistent with Toxic Epidermal Necrolysis. Her clinical history was significant for exposure to a medication while in El Salvador, which was assumed to be alloupurinol for presumed gout flare, 2 weeks before presentation. Prior to IVIG treatment, labs findings demonstrated a positive ANA, high titer ENA with anti-SSA, anti-SSB presence, and low titer RF with uric acid level of 3.8. Her hospital course was complicated by pneumonia, bacteremia, prolonged wound healing, enteral feeding intolerance, and persistent musculoskeletal pain with ulnar neuropathy and right foot drop. She responded to stress dose steroids leading to a long steroid taper based on suspicion of a potential autoimmune source. On HD 86, she was finally showing signs of wound closure We obtained a rheumatology consult that identified the presence of likely Sjogren’s’ disease. With this diagnosis, we were able to start targeted rheumatologic therapy and ensure appropriate medical follow up at discharge.

Discussion: Patients with auto-immune diseases are at risk for skin disorders that mimic SJS or TEN, and the presence of undiagnosed auto-immune diseases can complicate and prolong treatment. An auto-immune diagnosis during the acute phase is difficult due the non-specific nature of many auto-immune laboratory markers, also found in critically ill patients. While Sjogren’s has been shown in limited literature to be a potential long-term complication of having SJS/TEN, the diagnosis in the acute setting was critical in targeting therapy for long-term care.