Drooling as a Red Flag: Insights From a Case Series in Severe Dermatomyositis With Literature Review

Dermatomyositis (DM) is a rare idiopathic inflammatory disease characterized by progressive proximal muscle weakness and distinctive dermatologic manifestations. Although dysphagia is a recognized complication of oropharyngeal muscle involvement, drooling (sialorrhea) is infrequently described and may be a marker of severe disease. This case series reports three adult patients with severe DM who presented with drooling, highlighting diagnostic challenges and therapeutic dilemmas in the management of refractory disease. All patients in the present study exhibited severe DM with prominent dysphagia, leading to drooling. Despite intensive immunosuppressive therapy, including high-dose corticosteroids, immunosuppressants, intravenous immunoglobulin, and Rituximab, the outcome was disappointing, highlighting the refractory nature of the disease. The patients experienced readmissions following the worsening of dysphagia and drooling, which eventually led to complications like aspiration pneumonia and sepsis and ultimately fatal outcomes. Anti-Ro52 and Anti-Nuclear Matrix Protein 2 (NXP2) antibodies correlated with a more severe disease course and poor prognosis. Although rare, drooling in DM may be a clinical marker of severe DM with extensive oropharyngeal involvement and an increased risk for life-threatening complications. Early evaluation and initiation of intensive combination therapy could improve patient outcomes. Further research is needed to refine treatment protocols for this challenging manifestation of DM patients.