12-year-old girl who had been diagnosed with Moebius syndrome a year earlier presented for evaluation of progressive tongue hemiatrophy that was causing her difficulty with eating and speaking. Magnetic resonance imaging (MRI) of the brain demonstrated tongue hemiatrophy without involvement of the hypoglossal nerve. A tongue biopsy showed atrophy of skeletal muscle, variably sized nerves, stromal calcification, mild hyperplasia of overlying mucosa, parakeratosis and mild subepithelial inflammation. The patient had forehead and post-auricular skin discoloration.
She was diagnosed with Parry-Romberg syndrome and en coup de sabre localized scleroderma. This rare complication of scleroderma can result in cosmetic disfigurement and functional impairments with speech and eating if diagnosis is delayed.
Lauren Harper, MD, is a rheumatology fellow at Nationwide Children’s Hospital, Columbus, Ohio. She earned her medical degree at Loma Linda University School of Medicine, California and completed her residency at Loma Linda University Medical Center.
About the Contest
The Rheumatology Image Library is a highly accessed teaching resource. The 2022 Image Competition sought images representing a diverse range of patients who show either characteristic or unusual manifestations of rheumatic disease, including systemic sclerosis, localized scleroderma and scleroderma mimics. In addition to the Best Overall Image, winning images were selected from five regions across the world.
Here, we present the image featured from North America. Look for the People’s Choice and other regional winners in future issues or in the Rheumatology Image Library.