Scleroderma dermatomyositis overlap syndrome

Abstract

A young female presented with oligoarthritis of the right hand, painful symmetrical quadriparesis, and darkening skin for 7 months. General physical examination showed hyperpigmentation on the face, neck, upper extremities, calcinosis cutis with swelling and tenderness in the right wrist, and interphalangeal joints with restriction of movements. Neurological examination revealed power in both upper limbs of 3/5 and 4/5 in both lower limbs, all deep tendon reflexes were diminished, and bilateral plantar was flexors. Investigations revealed creatinine phosphokinase – 3991 U/L, lactate dehydrogenase – 1009 mg/dL, antinuclear antibodies by enzyme-linked immunosorbent assay showed anticentromere antibodies positive, and anti-Mi2 positive. Electromyography was suggestive of myopathy. A muscle biopsy confirmed the diagnosis of dermatomyositis (DM). Hence, the diagnosis of limited scleroderma DM overlap syndrome was made. Serial electrocardiography showed sinus bradycardia and ventricular premature ectopics followed by sinus rhythm. She was treated with pulse therapy steroids, hydroxychloroquine, sulfasalazine, antiplatelets, and isoprenaline for cardiac involvement, with improvement in her symptoms.