Systemic sclerosis is a chronic systemic disorder of unknown etiology. Systemic sclerosis is characterized by thickening of the skin (scleroderma) and distinctive involvement of multiple internal organs, most notably the lungs, gastrointestinal tract, heart, and kidneys. The early stage of the disease, associated with prominent inflammatory features, is followed by the development of widespread functional and structural alterations in multiple vascular beds and progressive visceral organ dysfunction due to fibrosis (i.e excessive formation of fibrous connective tissue). The presence of thickened skin (scleroderma) distinguishes Systemic sclerosis from other connective tissue diseases.
Causes, incidence, and risk factors
The cause of scleroderma is unknown. People with this condition have a buildup of a substance called collagen in the skin and other organs. This buildup leads to the symptoms of the disease.
The disease usually affects people 30 to 50 years old. Women get scleroderma more often than men do.
Widespread scleroderma can occur with other autoimmune diseases, including systemic lupus erythematosus and polymyositis. In such cases, the disorder is referred to as mixed connective disease.
Some types of scleroderma affect only the skin, while others affect the whole body.
Localized scleroderma usually affects only the skin on the hands and face. It develops slowly, and rarely, if ever, spreads throughout the body or causes serious complications.
Systemic scleroderma, or sclerosis, may affect large areas of skin and organs such as the heart, lungs, or kidneys. There are two main types of systemic scleroderma: Limited disease (CREST syndrome) and diffuse disease.
Skin symptoms of scleroderma may include:
Fingers or toes that turn blue or white in response to hot and cold temperatures (See: Raynaud's phenomenon)
Hair loss
Skin hardness
Skin that is abnormally dark or light
Skin thickening, stiffness, and tightness of fingers, hands, and forearm
Small white lumps beneath the skin, sometimes oozing a white substance that looks like toothpaste
Sores (ulcers) on the fingertips or toes
Tight and mask-like skin on the face
Bone and muscle symptoms may include:
Joint pain
Numbness and pain in the feet
Pain, stiffness, and swelling of fingers and joints
Wrist pain
Breathing problems may result from scarring in the lungs and can include:
Dry cough
Shortness of breath
Wheezing
Digestive tract problems may include:
Bloating after meals
Constipation
Diarrhea
Difficulty swallowing
Esophageal reflux or heartburn
Problems controlling stools (fecal incontinence)
There is no specific treatment for scleroderma in conventional system(Allopathy) of medicine.
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