Dermatomyositis a Vocal and Swallowing Disease Entity

The otolaryngologist must consider the diagnosis of dermatomyositis when he examines patients who complain of hoarseness, dysphagia or have symptoms referable to the tongue or upper esophageal and laryngeal musculature. It is the rarest of the so‐called collagen diseases. There is a 50% mortality rate within the first two years but spontaneous remissions are seen. Dermatomyositis is a collagen disease in which polymyositis with creatinuria and accompanying dermatitis and periorbital “heliotrope” edema are the distinguishing characteristics. No other collagen disease has such a strikingly consistent creatinuria. Dermatomyositis may follow an acute infectious process or be related to a cancer process. Of dermatomyositis patients, 17% to 50% will demonstrate a malignant process. Treatment of the malignancy may be followed by remarkable improvement in the dermatomyositis. The polymyositis usually causes dysphagia, dysphonia and tongue weakness. Laboratory tests are not of much help in diagnosis. Diagnoses depends upon muscle biopsy. Urine creatine is increased and creatinine is decreased. Lactic dehydrogenase, transaminase, aldolase and creatine phosphokinase with isoenzymes are increased secondary to the muscle degenerative process. Electromyography is helpful in determining biopsy site. Radiologic findings show diseased portions of the esophagus to gape (“the vallecular sign”) and bolus propulsion to be defective. Cinefluoroscopy and esophageal motility studies show absent esophageal motility. Muscle biopsies show parenchymal and interstitial changes of nonspecific character and degree. Primarily, the disease must be differentiated from disseminated lupus erythematosus and scleroderma. Prednisone is the drug of choice for treatment using a descending dosage scheduled according to the patient's objective improvement and serum enzyme studies. In resistant cases, immuno‐suppressive agents such as methotrexate have been effective. Other agents have been useful. The etiology of the disease is unknown. Picornaviruses and other viruses have been implicated. Current knowledge considers dermatomyositis to be a disease in which an autoimmune response to an antigen causes muscle damage with specific cytotoxicity of muscle cells being produced by the action of lymphocytes and the release of lymphotoxin.