What is systemic sclerosis (SSc)?
What is systemic sclerosis (SSc)?
Systemic sclerosis (SSc), is an autoimmune disorder, characterized by alterations of humoral and cellular immunity, leading to fibroproliferative alterations in the microvasculature which in turn causes excessive deposition of collagen fibers in the skin and internal organs [1,2].
Is it possible to have systemic sclerosis without thickening of the skin?
It is possible to have Systemic Sclerosis without skin thickening (sine scleroderma), which is when a patient has the internal organ manifestations without the skin findings, but this is very rare.
What are the signs and symptoms of systemic sclerosis (SMS)?
Key findings in systemic sclerosis include skin and joint changes, Raynaud phenomenon, and esophageal changes, but life-threatening effects may involve organs such as the lungs, heart, or kidneys.
What is systemic scleroderma and how is it characterized?
Systemic scleroderma is an autoimmune disorder that affects the skin and internal organs. It is characterized by the buildup of scar tissue (fibrosis) in the skin and other organs.
Systemic sclerosis (SSc) is an autoimmune inflammatory condition. It results in potentially widespread fibrosis and vascular abnormalities, which can affect the skin, lungs, gastrointestinal tract, heart and kidneys. The skin becomes thickened and hard ( sclerotic ).
How does limited systemic sclerosis affect the body?
Limited systemic sclerosis affects just the skin or mainly only certain parts of the skin and is also called CREST syndrome. People who have this type develop skin tightening (scleroderma) over the face, hands, forearms, lower legs, and feet.
What is the pathophysiology of systemic sclerosis?
Systemic sclerosis (SSc) is an autoimmune inflammatory condition. It results in potentially widespread fibrosis and vascular abnormalities, which can affect the skin, lungs, gastrointestinal tract, heart and kidneys. The skin becomes thickened and hard (sclerotic).
What is the difference between systemic sclerosis and sclerodactyly?
The extent of skin fibrosis defines ‘diffuse’ versus ‘limited’ systemic sclerosis. Sclerodactyly: thickening and tightness of the skin of the fingers (or toes). Can be spindle-shaped. Ulceration can lead to dry gangrene and eventual loss of the tips of the fingers (like frostbite).
What are the possible complications of systemic sclerosis sine scleroderma?
Interstitial lung disease and scleroderma renal crisis are the major complications. In systemic sclerosis sine scleroderma, patients have systemic sclerosis–related antibodies and visceral manifestations of the disease but no skin tightening. Immunologic mechanisms and heredity (certain human leukocyte antigen subtypes) play a role in etiology.
What is scleroderma (SSC)?
The term scleroderma literally means hard skin. But scleroderma (also known as systemic sclerosis, or SSc) is much more than a skin disorder. While nearly all persons with systemic sclerosis have issues with their skin, particularly excessive scarring, swollen, tight, or hard skin on their fingers, they also have organ system involvement.
