S4418 Unraveling Dermatomyositis: Severe Dysphagia and Cutaneous Findings in a 69-Year-Old Man

Introduction: Dermatomyositis is an idiopathic inflammatory myopathy characterized by proximal muscle weakness and pathognomonic skin findings. Dysphagia, which occurs in up to 40% of cases, can result from oropharyngeal muscle involvement and may present before muscle symptoms. We present a case of dermatomyositis with prominent cutaneous manifestations and severe dysphagia, emphasizing early recognition and immunosuppressive management. Case Description/Methods: A 69-year-old African American man initially presented to his primary care physician with a violaceous rash and mass on his left lower eyelid. A few days later, he developed a coalescing, pruritic rash on his chest, arms, and face, along with periungual erythema. A week after the rash presented, he reported progressive dysphagia to both solids and liquids, along with odynophagia and a 30-pound weight loss. Dermatology suspected dermatomyositis and performed a skin biopsy. He was admitted for worsening dysphagia and failure to thrive. Further workup revealed elevated creatine kinase (∼800 U/L), LDH, AST, and positive TIF-1γ antibodies. Despite equivocal proximal muscle weakness, his rash, lymphadenopathy, cytopenias, and dysphagia supported the diagnosis of dermatomyositis. Computed tomography imaging showed cervical and axillary lymphadenopathy; however biopsy was negative for malignancy. His esophagogastroduodenoscopy was unremarkable, but a modified barium swallow demonstrated severe oropharyngeal dysphagia with aspiration. The inpatient GI team was consulted for possible percutaneous endoscopic gastrostomy tube placement. However, it was decided to start therapy for dermatomyositis, and monitor if dysphagia improved. He was treated with IVIG, high-dose corticosteroids, and mycophenolate mofetil. His swallowing function improved significantly by day 2 of therapy and although not fully back to baseline, he resumed eating without aspiration events. Discussion: Dysphagia in dermatomyositis is frequently due to cricopharyngeal and oropharyngeal muscle dysfunction and may precede or occur in the absence of overt muscle weakness. The patient’s TIF-1γ positivity, associated with increased malignancy risk, warranted thorough malignancy screening. This case demonstrates the need to consider dermatomyositis in patients presenting with rapidly progressive dysphagia and cutaneous findings, even in the absence of clear muscle weakness. It may be prudent to monitor for improvement of dysphagia symptoms with immunosuppressive therapy, rather than rush to enteral feeding via percutaneous endoscopic gastrostomy in such cases.