The n e w e ng l a n d j o u r na l of m e dic i n e

Images in Clinical Medicine

Stephanie V. Sherman, M.D., Editor

Periorbital Edema in Dermatomyositis

A B

A
Caoilfhionn M. Connolly, M.D. 61-year-old woman presented to the rheumatology clinic with
Lisa Christopher-Stine, M.D., a 6-month history of periorbital swelling and shoulder weakness. She re-
M.P.H. ported no dyspnea, cough, or joint pain. The physical examination was
Johns Hopkins University School notable for erythema of the upper and lower eyelids and substantial periorbital
of Medicine edema (Panel A). There was also midfacial erythema involving the nasolabial folds,
Baltimore, MD poikiloderma of the upper back, and 4/5 strength of the shoulder abductors on
lchrist4@jhmi.edu
both sides. Laboratory testing showed a creatine kinase level of 6300 U per liter
This article was published on April 8, (reference range, 24 to 170), an antinuclear antibody titer of 1:320, and positivity
2023, at NEJM.org.
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 in-
flammatory 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 naso-
labial 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).
DOI: 10.1056/NEJMicm2210861
Copyright © 2023 Massachusetts Medical Society.

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