Periorbital Edema in Dermatomyositis
Caoilfhionn M. Connolly, Lisa Christopher-Stine
N Engl J Med 2023; 388:e52
DOI: 10.1056/NEJMicm2210861
A 61-year-old woman presented to the rheumatology clinic with a 6-month history of periorbital swelling and shoulder weakness. She reported no dyspnea, cough, or joint pain. The physical examination was notable for erythema of the upper and lower eyelids and substantial periorbital edema (Panel A). There was also midfacial erythema involving the nasolabial folds, poikiloderma of the upper back, and 4/5 strength of the shoulder abductors on both sides. Laboratory testing showed a creatine kinase level of 6300 U per liter (reference range, 24 to 170), an antinuclear antibody titer of 1:320, and positivity for anti-p155/140 myositis autoantibody. Magnetic resonance imaging of the right deltoid muscle showed muscle edema. A skin biopsy showed interface dermatitis. A diagnosis of dermatomyositis was made. Dermatomyositis is an idiopathic inflammatory myopathy characterized by immune-mediated muscle and skin injury. As in this case, the pathognomic skin finding of a periorbital heliotrope rash may be accompanied by eyelid edema in dermatomyositis. Other typical skin findings in this patient included shawl sign and a malar rash that did not spare the nasolabial folds. Treatment with oral glucocorticoids, methotrexate, and intravenous immune globulin was initiated. Screening for cancer was negative. At the 2-month follow-up, the patient’s weakness, rashes, and periorbital edema had abated (Panel B).
