For the 2023 Image Competition, the ACR sought images representing a diverse range of patients with idiopathic inflammatory myopathies (IIMs) or IIM mimics.
Chronic Facial Ulcers in Anti-Melanoma-Differentiation-Associated Gene 5 (MDA-5) Antibody Amyopathic Dermatomyositis
These images depict a 27-year-old patient who developed erythematous violaceous lesions over his upper chest, face and scalp over six months. Such lesions are associated with photosensitivity, non-scarring alopecia, oral ulcers, heliotrope rash and Gottron papules. Progressive ulcers over the bilateral malar areas and the left angle of the mandible followed. Ulcer edge biopsy showed perivascular and peri-adnexal lymphoid infiltrates, vacuolar interface dermatitis and basement membrane zone thickening, with no clinical, radiological or laboratory evidence of myositis, interstitial lung disease or arthritis.
The patient was positive for the anti-melanoma-differentiation-associated gene 5 (MDA-5) antibody and was diagnosed with amyopathic dermatomyositis. Anti-MDA-5 antibody is usually associated with digital and extensor ulcers; however, facial ulceration, as in this case, is rare.
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Anti-MDA-5 antibody positivity is associated with often severe and rapidly progressive interstitial lung disease (ILD). Thus, all patients with anti-MDA-5 antibody should be evaluated with pulmonary function testing and high-resolution computed tomography imaging of the chest. It’s important to carefully evaluate these patients for muscle involvement, which can be subtle.
For more information, visit https://www.the-rheumatologist.org/article/a-world-of-difference-updates-from-the-global-rheumatology-summit, and review the section on IIMs.
Rajat Ranka, MD, is a senior resident in rheumatology and clinical immunology at All India Institute of
Medical Science, Rishikesh.
About the Contest
The Rheumatology Image Library is a highly accessed teaching resource and images from the ACR’s annual contest are added to it, keeping the library dynamic and current. In addition to the Best Overall Image in 2023, winning images were selected from four regions around the world. Look for the People’s Choice and regional winners in future issues and on our website.