Recurrent Benign Esophageal Strictures in Tylosis: A Novel Clinical Association

Tylosis (Howel-Evans syndrome) is a rare autosomal dominant disorder characterized by palmoplantar hyperkeratosis and a markedly increased risk of esophageal squamous cell carcinoma. We report a 57-year-old man with palmoplantar hyperkeratosis, family history of palmoplantar hyperkeratosis and esophageal stricture, and progressive dysphagia found to have recurrent benign esophageal strictures requiring repeated endoscopic dilation. Serial biopsies were negative for eosinophilic esophagitis, dysplasia, or malignancy, and RHBDF2 sequencing revealed a variant of uncertain significance. This case suggests that recurrent benign strictures may represent an underrecognized manifestation of tylosis and highlights the importance of considering syndromic etiologies and maintaining close endoscopic surveillance.