Dermatomyositis (DM) is characterized by progressive proximal limb weakness and typical skin manifestations. The histological findings that show perifascicular atrophy and deposition of membrane attack complex are pathognomic features of DM. Dermatomyositis is categorized into classical DM and non-classical DM, which includes amyopathic DM and DM sine dermatitis. DM sine dermatitis is seldom described because of its rarity, making the diagnosis more challenging. We report a case of DM sine dermatitis, a rare phenotype of DM.
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Dermatomyositis is a rare and idiopathic inflammatory myopathy with a characteristic cutaneous manifestation. A 62-year-old female complained of polyarthralgia that lasted for many years. She was diagnosed with hypomyopathic dermatomyositis by the typical skin rash associated with dermatomyositis but without muscle involvement such as muscle weakness, elevated level of creatinine phosphokinase and aldolase. Her symptoms improved with treatment of hydroxychloroquine and prednisolone. We experienced a case of hypomyopathic dermatomyositis on 62-year-old female patient and report with review of literatures.
Dermatomyositis is characterized by progressive, symmetric, proximal muscle weakness and a nonsuppurative inflammatory myopathy of unknown etiology involving predominantly skeletal muscles. It is also characterized by typical skin lesions. Interstitial lung disease has a poor prognosis when it is associated with dermatomyositis. Organizing pneumonia is a disease in which granulation tissue fills the lumina of terminal and respiratory bronchioles and extends into the distal airspaces. The cryptogenic nature of the process is appreciated in that organizing pneumonia patterns of injury can be seen in secondary forms of the disease (secondary organizing pneumonia). Organizing pneumonia has been reported to occur in 5~10% in dermatomyositis-polymyositis patients. Anti-histidyl tRNA synthetase antibody (anti-Jo-1) is a predictive disease marker that is reported to occur in up to 70% of patients. We describe a 49-year-old male dermatomyositis patient who presented with organizing pneumonia and was found to have negative anti-Jo-1 antibody.