ACR Image Competition 2023 Results, Part 4

For the 2023 Image Competition, the ACR sought images representing a diverse range of patients with idiopathic inflammatory myopathies (IIMs) or IIM mimics.

Unilateral Heliotrope Rash in Anti-MDA-5 Antibody Dermatomyositis

These images depict a 36-year-old patient with arthralgias, dyspnea and a unilateral heliotrope rash (image A). A unilateral heliotrope rash is a distinctive, but rare, manifestation. Manual muscle testing was normal, but myositis was evident on magnetic resonance imaging scan (image B). A high-resolution computed tomography scan demonstrated peripheral groundglass opacities (image C). Capillaroscopy showed red blood cell extravasation and peri-capillary stippling (PCS; image D). Laboratory tests revealed anti-MDA-5 antibodies and normal muscle enzymes. The patient was diagnosed with anti-MDA-5 antibody dermatomyositis (DM).

Anti-MDA-5 antibody DM frequently presents various cutaneous manifestations, including ulcerations, alopecia and hyperkeratosis of the palms, also known as mechanic’s hands. The patient was treated with rituximab and mycophenolate mofetil, with clinical improvement and resolution of the PCS (image E).

Learn More

Many patients with anti-MDA-5 antibody-positive dermatomyositis have a high risk of pulmonary infection. When evaluating patients with anti-MDA-5 antibody-positive dermatomyositis, it may be necessary to repeat pulmonary function testing frequently to avoid missing disease progression. For more information, visit https://www.the-rheumatologist.org/article/a-world-of-difference-updates-from-the-global-rheumatology-summit/?singlepage=1, and review the section on IIMs.

Doron Rimar, MD, is a rheumatologist at Bnai Zion Medical Center, Haifa, Israel. His main research focus

is systemic sclerosis.