S4349 Esophageal Lichen Planus Masquerading as Lymphocytic and Eosinophilic Esophagitis: Treatment Response to Tofacitinib

Introduction: Lichen planus is a chronic, immune-mediated disorder that affects mucocutaneous sites including skin, mouth and esophagus. Esophageal lichen planus (ELP) is a rare, though increasingly emerging entity without standardized treatment. We describe herein the successful treatment of ELP with the Janus kinase (JAK) inhibitor tofacitinib. Case Description/Methods: A 53-year-old woman presented with worsening gastroesophageal reflux disease and dysphagia. Esophagogastroduodenoscopy (EGD) showed a narrow esophagus with white lacy friable mucosa. Esophageal biopsies showed 30 intraepithelial lymphocytes/high-power field (hpf) with minimal eosinophils (5 eos)/hpf. Lymphocytic esophagitis (LyE) was diagnosed. LyE progressed despite high-dose proton pump inhibitors (PPI) and oral viscous budesonide, requiring Savary dilation of the narrow-caliber esophagus to 30-Fr. Escalation to systemic steroid with prednisone 40 mg, while continuing high-dose PPI, failed to improve the working diagnosis of LyE. On high-dose esomeprazole and prednisone, EGD showed a diffusely narrowed 9-mm esophagus with diffuse patches of white lacy mucosa, multiple thick white linear exudates, edema, rings and strictures status post dilation. Esophageal biopsies showed prominent lymphocytosis but also 20 eos/hpf, making LyE now unlikely. Esophageal eosinophilia could suggest eosinophilic esophagitis or the eosinophilic esophagitis-variant with lymphocytosis. At this point, combining endoscopic and histologic findings, the thick white linear exudates, treatment refractory nature, review of esophageal biopsies revealed lichenoid esophagitis. Additional evaluation of persistent gingivitis unresponsive to steroids further supported the alternative diagnosis of ELP with oral involvement. Off label tofacitinib 5 mg twice daily was started. Gingivitis resolved within 1 month. Serial EGD’s demonstrated mucosal healing after 6 months on tofacitinib. Esophageal biopsies showed absent lymphocytosis with <10 eos/hpf. Narrow-caliber esophagus responded to serial dilations. Discussion: ELP is a rare but likely under-diagnosed disease. Treatment generally consists of immunosuppressive agents, however, is not standardized in part due to the overall rarity of the condition. Typical regimens include PPI and swallowed steroids initially, followed by systemic steroids and immunosuppressive agents. JAK inhibitors have shown promise in treating ELP in a few case reports. This case of ELP with response to tofacitinib adds to the encouraging literature on JAK inhibitors as a potential treatment option for patients with ELP.