Unusual Bilateral Upper Extremity Pitting Edema in a Patient With Severe Dermatomyositis

Abstract

A 56-year-old Hispanic female presented with six weeks of progressive dysphagia, proximal muscle weakness, erythematous rash, bilateral upper extremity pitting edema, and left lower extremity pitting edema. She had preserved heart function and a normal echocardiogram (ECG). She presented with elevated creatine kinase (CK) and aldolase, with normal renal function. Muscle biopsy suggested idiopathic polymyositis. No blood clot was seen on deep veinthrombosis (DVT) ultrasound. The myositis antibody panel showed the NXP-2 antibody, which is usually seen in pediatric dermatomyositiscases. In our literaturesearch, extremity pitting edema is an unusual way of presentation in dermatomyositis. She responded with intravenous immunoglobulin (IVIg) and high-dose intravenous steroids. We used azathioprine for remission maintenance; her rash recurred after tapering steroids. We resumed tapering steroid therapy and started her on weekly methotrexate along with daily azathioprine. With this combination therapy, her rash and muscle function improved. We successfully tapered her steroids. In our literature search, combination therapy with azathioprine and methotrexate was not reported. Our patient is tolerating this therapy very well.