The KIR genes associated with the disease (KIR2DL2 and 2DS4del) were analyzed with the following clinical manifestations: sclerodactyly, musculoskeletal damage, Raynaud's phenomenon, inflamed fingers, telangiectasia, digital ulcers, calcinosis, esophageal dysfunction, interstitial lung disease, and pulmonary arterial hypertension. The gene discussed is KIR3DL1; the disease is telangiectasis.