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A 55-year-old Hispanic woman presents to her family physician with a diffuse rash and increasing muscle weakness. The initial rash (without weakness) 2 months prior was thought to be a photosensitivity reaction to her new hydrochlorothiazide (HCTZ) prescription. She stopped the HCTZ and the rash initially improved with some topical corticosteroids. At the time of her current presentation, she had trouble getting up from a chair, walking, and lifting her arms over her head. The rash was prominent in sun-exposed areas, but was also seen in a shawl-like distribution in non–sun-exposed areas (Figure 189-1). Aside from her hypertension and obesity, the patient did not have any previous chronic medical conditions. She was afebrile with no other pertinent findings on physical exam.

FIGURE 189-1

Initial presentation of dermatomyositis in a 55-year-old Hispanic woman. Prominent violaceous erythema with scale is visible on the chest, face, and arms. Deep-red erythema is especially visible on the side of the face. The scalp is red and scaling. (Reproduced with permission from Richard P. Usatine, MD.)

This is a classic presentation of dermatomyositis with the typical rash and proximal muscle weakness. Close attention to the rash around her eyes demonstrates the pathognomonic heliotrope rash of dermatomyositis (Figures 189-2 and 189-3). The patient also has Gottron papules on the fingers, seen best in this case over the proximal interphalangeal (PIP) joint of the third finger (Figure 189-4). There was periungual erythema and ragged cuticles. The scalp was red and scaly. Her neurologic exam was consistent with proximal myopathy. She also had some trouble swallowing bread; and dysphagia is not unusual in dermatomyositis. Laboratory tests showed mild elevations in muscle enzymes with the aspartate transaminase (AST) having the greatest elevation. In other cases, the creatine kinase (CK) can be very elevated.

FIGURE 189-2

Close-up of the heliotrope (violaceous) rash around the eyes of the patient in Figure 189-1. (Reproduced with permission from Richard P. Usatine, MD.)

FIGURE 189-3

View showing the bilateral heliotrope rash of the patient in Figure 189-1. A pathognomonic sign of dermatomyositis. (Reproduced with permission from Richard P. Usatine, MD.)

FIGURE 189-4

Hand involvement showing two Gottron papules over the knuckles (arrows) and erythematous nail folds (periungual erythema) in the patient in Figure 189-1. (Reproduced with permission from Richard P. Usatine, MD.)

The family physician started the patient on 60 mg of prednisone daily and topical steroids for the affected areas. The patient responded well to prednisone and 2 weeks later ...

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