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A teenage girl presents with a new rash on her face and hands for the past few months. In addition to going to school she works as a waitress and has noted that it is harder to carry heavy trays. She also has gum inflammation and is wondering if this could be related to everything else. The physician notes the heliotrope rash around her eyes (Figure 174-1) and the Gottron papules on the dorsum of her knuckles (Figure 174-2). He considers that this may be dermatomyositis and tests for proximal muscle weakness. The proximal muscles are not found to be weak on physical exam although a subsequent blood test showed a mildly elevated CK and AST. The physician uses his dermatoscope to look at the nail folds and sees many dilated capillary loops (Figure 174-3). On the oral examination, there is a marginal gingivitis and the dermatoscope shows a similar dilated capillary pattern around the tooth. A diagnosis of dermatomyositis is made. The patient was treated with prednisone and hydroxychloroquine and improves greatly. The patient was then tapered off the prednisone fully with no relapse.
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Juvenile dermatomyositis is a rare, idiopathic inflammatory disease involving the striated muscles and the skin. Similar to adult cases of dermatomyositis, the disease is primarily characterized by progressive, symmetrical, proximal muscle weakness. Dermatologic manifestations may occur with or without muscular disease and include the heliotrope rash (Figures 174-1 174-4, and 174-5), “shawl sign,” and Gottron papules over the finger joints (Figures 174-4 to 174-7). Although primarily a disease of muscle and skin, juvenile dermatomyositis has a clear association with myocarditis, vasculitis, calcinosis and interstitial lung disease.1 Unlike adult dermatomyositis, ...