Refractory Oral Lichenoid lesion Associated with Dermatomyositis Resolved by Intravenous Immunoglobulin Therapy

Tanaka, Takako I., Paul C. Edwards, and Shin-Mey RY. Geist

Dermatomyositis (DM) is a chronic immune dysfunction of unknown etiology.  It causes inflammatory changes in the skeletal muscles and has pathognomonic and characteristic skin lesions. Skin manifestations usually appear early in the disease process.  Proximal muscle weakness can progress to multiple system involvement including cardiovascular and pulmonary dysfunction. The observation that approximately 20% to 25% of adult patients have a hidden internal malignancy makes early diagnosis crucial. Recent advances in immunotherapy have greatly reduced the mortality and morbidity, and improved the quality of life of DM patients.  Both the skin and muscle lesions are highly relevant to oral healthcare providers as they have great significance in aiding diagnosis and management of the disease as well as indicating disease progress.  Oral mucosal involvement and muscle weakness of the orofacial region also contribute to the challenge of oral healthcare. We report a case of a patient whose skin lesion was misdiagnosed as systemic lupus erythematosus for 4 years, in spite of complaints of proximal muscle weakness and systemic corticosteroids and immunosuppressants were not effective.  His oral lesions were likewise overlooked for two years. The patient reported regaining muscle strength after  two courses of IVIg therapy. On follow-up examination, the oral lesions which was diagnosed as lichenoid mucositis had completely resolved.