Laryngeal edema in a patient with dermatomyositis

Abstract Dermatomyositis (DM) is a multisystem connective tissue disease, characterized by inflammatory changes and degeneration of the skin and muscle. Laryngeal edema coinciding with DM is quite rare. We report a DM case with laryngeal edema that was first suspected to be caused by hypopharyngeal carcinoma but as then considered to represent a unique symptom preceding DM. A 61-year-old man with neck swelling and edema of the left arytenoid was referred to our institute. Since hypopharyngeal carcinoma was suspected, a biopsy for the left hypoparynx was performed, but the results showed an inflamed squamous epithelium with mild basal cell hyperplasia. After the appearance of muscle weakness extending to the proximal limbs and elevations in the serum CK and ALD levels, DM was suspected. MRI findings of the left upper rim and the result of n -EMG finally led to the diagnosis of DM. To the best of our knowledge, this is a second reported case of laryngeal edema occurring as a symptom that preceded DM. Although inflammation of the adjacent muscle and excessive vascular permability in subcutaneous tissue, due to DM, may be a cause of laryngeal edema, it is also possible that unknown causes related to DM lead to laryngeal edema, which is a novel, unique feature that distinguishes this case from typical DM cases.