S2707 Disappearing Bile Ducts and Emerging Hemophagocytosis in a Patient With Toxic Epidermal Necrolysis

Abstract

INTRODUCTION: Vanishing bile duct syndrome (VBDS) is an uncommon condition of progressive bile duct loss, which often presents with immuno-allergic signs and can be fatal or necessitate liver transplantation. Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare spectrum of immune complex-mediated mucocutaneous reactions which share common immuno-allergic features and triggers with VBDS. Multiple previous case reports have described the co-occurrence of SJS/TEN and VBDS that is more common in children. Described here is a case of a young adult male who developed TEN with VBDS and subsequent hemophagocytic lymphohistiocytosis (HLH) after taking ibuprofen for influenza B. CASE DESCRIPTION/METHODS: A 26-year-old previously healthy male presented to an outside hospital with a 5-day history of fevers, chills, dry cough, and sore throat and a subsequent 7-day history of worsening rash over his back, chest, and extremities. He reported significant ibuprofen use for 3 days before the onset of his rash. Upon presentation, the patient was found to be influenza B positive and was started on oseltamivir. Exanthematous eruptions covered approximately 20% body surface area and quickly progressed to 80%. Skin biopsy findings were consistent with TEN. He was transferred to our facility for specialized burn unit care and was treated for TEN with intravenous immunoglobulin and etanercept with gradual improvement of his rash. Liver biopsy on hospital day 30 for severe cholestatic liver injury demonstrated vanishing bile ducts. Alkaline phosphatase peaked to 2278 U/L on hospital day 44 and serum total bilirubin peaked at 49.2 mg/dL on hospital day 61. Fevers, tachypnea, tachycardia, and leukocytosis persisted, and suspected HLH was diagnosed following a bone marrow biopsy. At the time of abstract submission, the patient remains hospitalized receiving HLH treatment with etoposide and high-dose steroids. DISCUSSION: This case report highlights a concurrent case of SJS/TEN and vanishing bile duct syndrome following an episode of acute influenza B. While there have been previously reported cases of VBDS with concurrent SJS/TEN, this appears to be the first reported case of SJS/TEN, VBDS, and subsequent HLH in the same patient. While this is a single case, it raises the possibility of a shared immuno-allergic pathogenesis between these three conditions.Table 1.: Peak hepatic panel lab valuesTable 2.: Hemophagocytic Lymphohistiocytosis diagnostic criteria