Author Affiliations: Departments of Otolaryngology–Head and Neck Surgery (Drs Pepper, Kupfer, and Hogikyan) and Pathology (Dr McHugh), University of Michigan, Ann Arbor.
Systemic sclerosis (SSc), or systemic scleroderma, is an autoimmune disease characterized by excessive interstitial fibrosis throughout the body.1 The skin is most frequently affected, resulting in diffuse thickening known as scleroderma (“hard skin”). Common otolaryngologic manifestations include trismus, gastroesophageal reflux disease (GERD), and ankyloglossia, in addition to the aforementioned skin changes. One study reported that approximately 9% of patients with scleroderma have at least intermittent dysphonia.2 Laryngoscopic findings in patients with scleroderma may include a wide range of nonspecific changes associated with reflux laryngitis due to a patulous lower esophageal sphincter and distal third of the esophagus.3 More rarely, SSc has been associated with vocal fold paresis and cricoarytenoid joint fixation.4-7 This report describes a patient with SSc presenting with moderate to severe dysphonia due to bilateral submucosal vocal fold lesions. The histopathologic characteristics of these lesions demonstrate changes that are characteristic of SSc and that to date have not been well described for the vocal folds. Case data, surgical approach, and videostroboscopic findings are discussed.
Pepper J, Kupfer RA, McHugh JB, Hogikyan ND. Histopathologic Findings and Clinical Manifestations in a Patient With Dysphonia and Vocal Fold Involvement by Systemic Sclerosis. Arch Otolaryngol Head Neck Surg. 2011;137(8):816–819. doi:10.1001/archoto.2011.119
Browse and subscribe to JAMA Network podcasts!
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: