A 52-year-old woman comes to the office due to a diffuse rash that began a few weeks ago. Lately, the patient has also been having difficulty rising from a seated position and climbing stairs. Vital signs are within normal limits. The patient has an erythematous rash on the upper chest and violaceous periorbital edema. Examination shows symmetric proximal muscle weakness in the legs. This patient's condition is most often associated with which of the following?

Question

A 52-year-old woman comes to the office due to a diffuse rash that began a few weeks ago.  Lately, the patient has also been having difficulty rising from a seated position and climbing stairs.  Vital signs are within normal limits.  The patient has an erythematous rash on the upper chest and violaceous periorbital edema.  Examination shows symmetric proximal muscle weakness in the legs.  This patient's condition is most often associated with which of the following?

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Explanation: 11ec25c8_d516_2d37_bc81_85153424b3c6_MD0003_00 Dermatomyositis is an autoimmune condition that is twice as common in women. In its most classic form, it causes a proximal muscle inflammatory myositis and characteristic cutaneous findings (eg, violaceous poikiloderma over various regions of the body). On the face, the eruption is often accompanied by periorbital erythema (often with edema) and is known as the heliotrope sign. The erythematous rash follows a photodistributed pattern on the chest (v sign) and back (shawl sign). Violaceous, occasionally scaly, lesions that can be flat (Gottron sign) or raised (Gottron papules) can be present on the metacarpophalangeal joints, proximal interphalangeal joints, elbows, and knees and are pathognomonic for dermatomyositis. Due to the inflammatory nature of dermatomyositis, patients have elevated creatine phosphokinase (CPK) that is generally 10 times the upper limit of normal but can vary. The classic autoantibodies associated with dermatomyositis are anti-Mi-2 (against helicase) and anti-Jo-1 (antisynthetase antibody, found more commonly in polymyositis). Malignancies are more common in patients with dermatomyositis (>15% of adult patients). The most common malignancies are adenocarcinomas (eg, ovarian, lung, pancreatic, stomach, colorectal cancers). The pathophysiology for this association is unclear but may be related to cross-reactive autoantigens (eg, in the muscles and on tumor cells). Regular, age-appropriate cancer screening is essential, and other studies (eg, chest x-ray, gynecologic evaluation) are often considered at initial diagnosis for further investigation of underlying malignancy. (Choice A) Alveolar hemorrhage can be caused by autoimmune diseases (eg, ANCA-associated vasculitides and lupus) and toxins (eg, cocaine, amphetamine). Dermatomyositis is associated with interstitial lung disease (10% of patients). (Choice B) Aortic aneurysms can be caused by autoimmune diseases (eg, Takayasu arteritis, giant cell arteritis), infections (eg, syphilis, salmonella), and connective tissue disorders (eg, Marfan, Ehlers-Danlos). (Choice C) Carpal tunnel syndrome is associated with autoimmune conditions (eg, rheumatoid arthritis), infiltrative diseases (amyloidosis), and endocrinopathies (eg, diabetes, hypothyroidism). (Choice D) Glomerulonephritis can be caused by infections (eg, hepatitis B, hepatitis C, HIV), protein deposition diseases (eg, amyloidosis), and autoimmune diseases (eg, lupus, ANCA-associated vasculitides). Rarely, the elevated CPK in dermatomyositis can cause a myoglobin-induced acute kidney injury from tubular injury (rather than glomerulonephritis). (Choice F) Autoimmune diseases associated with sudden visual loss include giant cell arteritis and multiple sclerosis. Educational objective: Dermatomyositis is typically characterized by cutaneous findings accompanied by proximal muscle weakness. Over 15% of adult patients will have or develop a malignancy, most commonly adenocarcinomas. Regular, age-appropriate cancer screening is essential.

A 52-Year-Old Woman Comes to the Office Due to a Diffuse