A 15-y-old girl presented with progressive skin stiffening and pigmentary changes for 4 mo. There was also proximal muscle weakness, Raynaud’s phenomenon, and dysphagia for solids. Examination revealed salt-and-pepper skin pigmentation over elbow, interphalangeal, and metacarpophalangeal joints, diffuse skin thickening, sclerodactyly, Mauskopf face, and abnormally dilated capillaries in nailfold capillaroscopy (Fig.
1). Muscle MRI showed myositis and muscle atrophy, with elevated creatinine kinase. Echocardiography showed mild pulmonary hypertension with normal CT chest and barium swallow. ANA was positive (1:250 speckled pattern) but antibodies were negative against dsDNA, PM-Scl, Scl-70, RNP-3, SS-A & B, Ro-52, Jo-1, Ku, Centromere, and Mitochondrial M2. A diagnosis of diffuse cutaneous systemic sclerosis (SSc) with inflammatory myopathy was made based on ACR-EULAR criteria (score = 15). She was started on pulse steroids followed by maintenance methotrexate, oral nifedipine, and proton pump inhibitor. On follow-up, there were improvements in weakness and skin changes. …
