Although SSc can affect almost any part of the body, skin disease is the most consistent clinical manifestation. Skin disease typically starts in the hands with an edematous phase of hand swelling lasting one to several months. The skin then progressively thickens and tethers to underlying tissues. In diffuse cutaneous SSc (dcSSc), skin thickening, induration and tethering typically extend proximally up the arm and can involve the torso, abdomen, face and legs. Patients with limited cutaneous SSc (lcSSc) have skin disease limited to below the elbow and face and neck as well as other characteristic clinical features. SSc skin pathology (diffuse and limited cutaneous SSc) shows fibrosis and variable perivascular lymphocyte infiltration in the deep reticular dermis.