Review Question - QID 102766

A 39-year-old woman with a rash around her eyes (heliotrope rash), along with a rash on her shoulders (shawl sign), elevated CK, ESR, and ferritin, in the setting of proximal muscle weakness is most likely suffering from dermatomyositis.

Dermatomyositis is an inflammatory myopathy with characteristic skin manifestations involving the eyes, the upper neck and shoulders, and the dorsum of hands. It has an association with other connective tissue disorders along with a higher risk of future and concurrent malignancy. Patient management includes long-term physical therapy along with antihistamines, sunscreen for the rashes, and oral corticosteroids for musculoskeletal pain. The goal of therapy is typically to maintain function without significant side effects.

Koler et al. discuss the diagnosis and treatment of dermatomyositis. They state that the diagnosis of dermatomyositis typically involves skin lesions (discussed above), along with proximal muscle weakness, elevated levels of CK, ferritin (acute phase reactant), and ESR, muscle pain, and electromyography showing muscle changes. The note that anti-Jo-1 antibodies may also be positive in these patients. Lastly, they recommend that in patients with disease resistant to corticosteroids, immunotherapies such as methotrexate and cyclophosphamide may be used.

The Muscle Study Group discusses a randomized controlled trial involving etanercept, a TNF-alpha inhibitor, as a mode of therapy for dermatomyositis. In their trial, they note that 5 of 11 subjects in the etanercept arm were successfully weaned off prednisone, while 0 of the 5 subjects in the placebo arm were able to be weaned off. They also state that the median of the average prednisone dosage after week 24 was 29.2 mg/day in the placebo group and 1.2 mg/day in the etanercept group (p = 0.02).

Figure A shows a symmetric "cloak-like" rash involving shoulders, neck, and possibly the chest wall. It is denoted as the "shawl sign" and is common in dermatomyositis.

Incorrect Answers:
Answer 1: Inclusion body myositis typically affects distal forearm and arm muscles and may cause bulbar symptoms of dysphagia.
Answers 2 and 3: The patient may have corticosteroid or statin induced myopathy, but her heliotrope rash (around the eyes) and the shawl sign are more typical of an inflammatory myositis.
Answer 5: Polymyositis also commonly affects proximal muscles and results in weakness, but it more commonly affects the lungs and the GI tract. It also does not present with a rash.