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Patient Story

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.

FIGURE 174-1

Classic heliotrope rash around the eyes of this teenager newly diagnosed with dermatomyositis. The color “heliotrope” is a pink-purple tint named after the color of the heliotrope flower. As expected, her heliotrope rash is bilaterally symmetrical. This rash resolved with prednisone and hydroxychloroquine. (Used with permission from Richard P. Usatine, MD.)

FIGURE 174-2

Hand involvement in the teenager in Figure 174-1 with Gottron papules over the finger joints. She has nailfold erythema and ragged cuticles (Samitz sign). (Used with permission from Richard P. Usatine, MD.)

FIGURE 174-3

A. Dilated nailfold capillary loops visible with dermoscopy in a teenager with newly diagnosed dermatomyositis. B. Marginal gingivitis in the same teen with newly diagnosed dermatomyositis. C. She also had dilated capillary loops on the gingival borders of her teeth seen with dermoscopy. The nailfold findings and gingival findings both resolved with treatment. (Used with permission from Richard P. Usatine, MD.)


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, ...

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