A 48-year-old previously healthy woman comes to the office due to progressively worsening muscle weakness for the past 2 months. The patient has difficulty with activities such as climbing stairs, getting up from chairs, and placing dishes in overhead cabinets. She also reports a 4.5-kg (10-lb) unintentional weight loss and occasional abdominal discomfort over the same interval. Physical examination shows weakness of the shoulder and hip girdle muscles. Other examination findings are shown in the image below. Further evaluation of this patient is most likely to reveal which of the following associated conditions?

Question

A 48-year-old previously healthy woman comes to the office due to progressively worsening muscle weakness for the past 2 months.  The patient has difficulty with activities such as climbing stairs, getting up from chairs, and placing dishes in overhead cabinets.  She also reports a 4.5-kg (10-lb) unintentional weight loss and occasional abdominal discomfort over the same interval.  Physical examination shows weakness of the shoulder and hip girdle muscles.  Other examination findings are shown in the image below.  Further evaluation of this patient is most likely to reveal which of the following associated conditions?

Quizplus · Accepted Answer

Dermatomyositis is a systemic autoimmune disease characterized by proximal muscle weakness resembling polymyositis, with additional inflammatory features involving the skin. Specific skin findings include the heliotrope rash in the periorbital area and cheeks and Gottron papules, which are raised erythematous plaques over the joints and bony prominences of the hands (as seen in this patient). Muscle biopsy is diagnostic and shows mononuclear perifascicular inflammation and atrophy (ie, occurring along the periphery of the fascicles). Dermatomyositis may occur alone or as a paraneoplastic syndrome of an underlying malignancy, most commonly due to underlying adenocarcinoma (eg, ovary, lung, pancreas). Symptoms may precede the diagnosis of malignancy but often parallel the course of the cancer. (Choice A) Hepatitis C infection is associated with porphyria cutanea tarda, which presents with skin fragility and a blistering rash in sun-exposed areas, and mixed cryoglobulinemic vasculitis, which presents with nonblanching palpable purpura. Muscle symptoms are not typical. (Choice C) Primary biliary cholangitis (previously primary biliary cirrhosis) is an autoimmune liver disease that can present with cholestasis, malabsorption, pruritus, and jaundice. Although the risk of this condition is increased in patients with autoimmune disease (eg, CREST syndrome, Sjögren syndrome), the association with dermatomyositis is not common. (Choice D) Tropheryma whippelii infection (Whipple disease) typically presents with arthritis, diarrhea, and fever. Patients may develop hyperpigmentation, but skin and muscle symptoms are otherwise not prominent. (Choice E) Type 2 diabetes is due to insulin resistance, which may also cause acanthosis nigricans, a condition characterized by velvety patches of brownish discoloration affecting the neck and skinfolds. Educational objective: Dermatomyositis is characterized by proximal muscle weakness resembling polymyositis, with additional inflammatory features involving the skin (heliotrope rash, Gottron papules). Both dermatomyositis and polymyositis may occur alone or as a paraneoplastic syndrome associated with an underlying adenocarcinoma (eg, ovary, lung, pancreas). __________ References: Dermatomyositis as the first manifestation of a lung tumor. (http://www.ncbi.nlm.nih.gov/pubmed/23602006) Malignancy in myositis. (http://www.ncbi.nlm.nih.gov/pubmed/21336621)

A 48-Year-Old Previously Healthy Woman Comes to the Office Due