The disease should be considered in those who present clinically with hardening, thickening and atrophy of the skin, or who also have lesions of the gastrointestinal tract, lungs, kidneys, heart, blood vessels and skeletal muscle system. Depending on the severity of the disease, in mild cases, only the skin of the fingers and face is involved, early manifestations are swelling of the face, hands and/or feet, tightening of the skin, inability to lift, like salami, also known as salami-like fingers, gradual thickening of the skin, loss of skin lines and folds, late skin hardening and atrophy, thin facial skin, dull expression, sharp nose, thinning of the ears, restricted mouth opening, like wearing a mask -like a mask. Most patients often have Raynaud’s phenomenon (a trichromatic change in which the fingers or toes turn white, then purple, and then red when exposed to cold or emotional stimuli) as the first symptom of the disease, and some patients have skin changes more than 10 years after Raynaud’s phenomenon. One-third of patients may have arthralgia and joint stiffness, and a few have definite arthritis. In addition, some patients present with severe muscle weakness, which is not easily distinguished from myositis. Some patients have progressive disease, often over a long period of time, some for decades, before clinical signs of specific visceral involvement appear. Severe scleroderma patients may have extensive skin changes involving the chest, abdomen, and back (diffuse type), and some patients describe “their stomach as a slab of stone” and may have visceral involvement.