A 65-year-old woman comes to the physician with a rash on her hands for the past 2 weeks. Two months ago she developed a rash on her face and forearms while vacationing at a beach resort. Her review of systems is positive for recent onset weakness of the shoulders and hips. She has no dysphagia, shortness of breath, fever, chills, or weight loss. She is a lifetime nonsmoker.
Her temperature is 37.2 C (99 F) , blood pressure is 134/82 mm Hg, pulse is 75/min, and respirations are 14/min. Her BMI is 21 kg/m². Cardiopulmonary examination is within normal limits. There is no hepatosplenomegaly. Range of motion of the spine is normal and without pain, scoliosis, or excessive kyphosis of the back. Upper and lower limb deep-tendon reflexes are 2+. She has difficulty rising from a chair. Muscle strength is 4/5 in both upper and lower extremities.
Laboratory results are as follows:

The rash is shown in the photograph.

This patient would require which of the following?

Question

A 65-year-old woman comes to the physician with a rash on her hands for the past 2 weeks. Two months ago she developed a rash on her face and forearms while vacationing at a beach resort. Her review of systems is positive for recent onset weakness of the shoulders and hips. She has no dysphagia, shortness of breath, fever, chills, or weight loss. She is a lifetime nonsmoker.
Her temperature is 37.2 C (99 F) , blood pressure is 134/82 mm Hg, pulse is 75/min, and respirations are 14/min. Her BMI is 21 kg/m². Cardiopulmonary examination is within normal limits. There is no hepatosplenomegaly. Range of motion of the spine is normal and without pain, scoliosis, or excessive kyphosis of the back. Upper and lower limb deep-tendon reflexes are 2+. She has difficulty rising from a chair. Muscle strength is 4/5 in both upper and lower extremities.
Laboratory results are as follows:

The rash is shown in the photograph.

This patient would require which of the following?

Quizplus · Accepted Answer

This patient's muscle weakness and elevated creatine kinase suggest a myopathic process. The characteristic rash in the hands (Gottron's papules located over the metacarpophalangeal joints) and history of photosensitive rash (shawl sign) suggest dermatomyositis as the most likely diagnosis. Up to 50% of patients may also present with violaceous periorbital rash in the upper eyelid (heliotrope rash). heliotrope rash Patients with inflammatory myopathy (dermatomyositis and polymyositis) are at fivefold increased risk of malignancy compared to the general population. Thus, screening should include the following: 1. Age-appropriate cancer screening tests (eg, mammogram and colonoscopy) in all patients 2. Additional tests in high-risk patients: CT of the chest, abdomen, and pelvisUrinalysis for hematuriaSerum CA125 and CA19-9Serum prostate-specific antigenUpper gastrointestinal endoscopy CT of the chest, abdomen, and pelvis Urinalysis for hematuria Serum CA125 and CA19-9 Serum prostate-specific antigen Upper gastrointestinal endoscopy (Choice A) This patient's rash on the hands and muscle weakness with elevated CK are not characteristic of systemic lupus erythematosus (SLE). SLE can cause myalgias and is somewhat less likely (<15%) to present in a patient of this age. (Choice C) Optic neuritis is rarely associated with systemic inflammatory disorders (eg, SLE and Wegener's granulomatosis). Patients typically present with painful unilateral vision loss over several hours to days. Funduscopic examination may be normal or show visible optic nerve inflammation. (Choice D) Liver biopsy may be indicated in patients with elevated transaminase levels, but this patient likely has a muscular source of the elevated enzymes due to inflammatory muscle disease. (Choice E) High-dose steroids (eg, 1 mg/kg/day to a maximum dose of 80 mg/day) for several months are usually indicated for treating dermatomyositis to achieve disease control prior to tapering to the lowest dose tolerated by the patient. Initial low-dose steroids are frequently used for polymyalgia rheumatica. Educational objective: Individuals with dermatomyositis and polymyositis have an increased risk of malignancy compared to the general population. Age-appropriate screening (eg, mammogram and colonoscopy) should be performed in these patients, and additional testing may be needed based on history and clinical symptoms. __________ References: Incidence, cancer risk and mortality of dermatomyositis and polymyositis in Taiwan: a nationwide population study. (http://www.ncbi.nlm.nih.gov/pubmed/21895620) Polymyositis, dermatomyositis and malignancy: a further intriguing link. (http://www.ncbi.nlm.nih.gov/pubmed/20026430) Incidence and predictive factors for malignancies in 136 Japanese patients with dermatomyositis, polymyositis and clinically amyopathic dermatomyositis. (http://www.ncbi.nlm.nih.gov/pubmed/20922453)

A 65-Year-Old Woman Comes to the Physician with a Rash