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Bilateral proximal muscle weakness Characteristic cutaneous manifestations in dermatomyositis (Gottron s papules, heliotrope rash) Diagnostic tests: elevated creatine kinase, muscle biopsy, electromyography Increased risk of malignancy, particularly in dermatomyositis Inclusion body myositis can mimic polymyositis but is less responsive to treatment
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Polymyositis and dermatomyositis are systemic disorders of unknown cause whose principal manifestation is muscle weakness Although their clinical presentations (aside from the presence of certain skin findings in dermatomyositis, some of which are pathognomonic) and treatments are similar, the two diseases are pathologically quite distinct They affect persons of any age group, but the peak incidence is in the fifth and sixth decades of life Women are affected twice as commonly as men, and the diseases (particularly polymyositis) also occur more often among blacks than whites There is an increased risk of malignancy in adult patients with dermatomyositis Indeed, up to one patient in four with dermatomyositis has an occult malignancy Malignancies may be evident at the time of presentation with the muscle disease but may not be detected until months afterward in some cases Rare patients with dermatomyositis have skin disease without overt muscle involvement, a condition termed dermatomyositis sine myositis
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Polymyositis may begin abruptly, but the usual presentation is one of gradual and progressive muscle weakness The weakness chiefly involves proximal muscle groups of the upper and lower extremities as well as the neck Leg weakness (eg, difficulty in rising from a chair or climbing stairs) typically precedes arm symptoms In contrast to myasthenia
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tein electrophoresis, and urinalysis, and should include ageand risk-appropriate cancer screening tests Given the especially strong association of ovarian carcinoma and dermatomyositis, pelvic ultrasonography and CA-125 levels may be useful in women The cost effectiveness of searching for occult malignancies in dermatomyositis by performing CT scanning of the chest, abdomen, and pelvis is not clear No matter how extensive the initial screening, some malignancies will not become evident for months after the initial presentation
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gravis, polymyositis and dermatomyositis do not cause facial or ocular muscle weakness Pain and tenderness of affected muscles occur in one-fourth of cases, but these are rarely the chief complaints About one-fourth of patients have dysphagia In contrast to scleroderma, which affects the smooth muscle of the lower esophagus and can cause a sticking sensation below the sternum, polymyositis or dermatomyositis involves the striated muscles of the upper pharynx and can make initiation of deglutition difficult Muscle atrophy and contractures occur as late complications of advanced disease Clinically significant myocarditis is uncommon even though there is often creatine kinase-MB elevation Patients who are bed-bound from myositis should be screened for respiratory muscle weakness that can be severe enough to cause CO2 retention and to require mechanical ventilation In dermatomyositis, the characteristic rash is dusky red and may appear in a malar distribution mimicking the classic rash of SLE Facial erythema beyond the malar distribution is also characteristic of dermatomyositis Erythema also occurs over other areas of the face, neck, shoulders, and upper chest and back ( shawl sign ) Periorbital edema and a purplish (heliotrope) suffusion over the eyelids are typical signs Periungual erythema, dilations of nailbed capillaries, and scaly patches over the dorsum of PIP and MCP joints (Gottron s sign) are highly suggestive Scalp involvement by dermatomyositis may mimic psoriasis Infrequently, the cutaneous findings of this disease precede the muscle inflammation by weeks or months Diagnosing polymyositis in patients over age 70 years can be difficult because weakness may be overlooked or attributed erroneously to idiopathic frailty Polymyositis can remain undiagnosed or will be misdiagnosed as hepatitis because of elevations in alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels A subset of patients with polymyositis and dermatomyositis develop the antisynthetase syndrome, a group of findings including inflammatory arthritis, Raynaud s phenomenon, interstitial lung disease, and often severe muscle disease associated with certain autoantibodies (eg, anti-Jo1 antibodies)
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