THE CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES Dermatomyositis and Granulomatous Myopathy Associated with Sarcoidosis

A previously healthy, 21-year-old female presented with acute dermatomyositis. Chest X-ray revealed bilateral hilar adenopathy, and mediastinal lymph node biopsy demonstrated noncaseating granulomata compatible with sarcoidosis. The patient improved spontaneously. The significance of bilateral hilar adenopathy in association with dermatomyositis and implications for management are discussed. RESUME: Dermatomyosite et myopathic granulomateuse associees a la sarcoidose Une femme agee de 21 ans, anterieurement en bonne sante, s'est presentee avec une dermatomyosite aigue. La radiographie pulmonaire a montre une adenopathie hilaire bilaterale et la biopsie d'un ganglion lymphatique mediastinal a montre une granulomatose non caseeuse compatible avec une sarcoidose. L'etat de la patiente s'est ameliore spontanement. Nous discutons de la signification de l'adenopathie hilaire bilaterale associee a la dermatomyosite et de la conduite a suivre dans de tels cas. Can. J. Neurol. Sci. 1988; 15: 426-429 A diagnosis of dermatomyositis (DM) can be made with certainty when the following five criteria are present: 1. weakness, 2. an electromyogram (EMG) which shows myopathic features plus fibrillations, 3. an elevated creatine kinase (CK), 4. a muscle biopsy which shows an inflammatory myopathy, and 5. the typical skin rash. An association between DM and malignancy is recognized. Sarcoidosis is a systemic disease of unknown etiology characterized by non-caseating granulomata involving multiple organs and almost invariably the lung. In this report, we describe a patient with DM and hilar adenopathy. The cooccurrence of bilateral hilar adenopathy and DM suggested malignancy, however mediastinal lymph node biopsy showed noncaseating granulomata. The possibility of an association between DM and sarcoidosis is discussed.