Scleroderma (Greek σκληρός skleros: hard, derma: skin) literally means hard skin.
There is a group of different rare diseases with an uncontrolled development of connective issue replacing skin alone or the skin and internal organs (especially the digestive tract, lungs, heart, and kidneys). Scleroderma is one of the so-called collagen diseases (a group of autoimmune connective tissue diseases).
To write a good research paper on scleroderma, you have to know that scleroderma is not curable, the disease can but be slowed down or stopped with medication and specialized rehabilitation. The former name of progressive systemic scleroderma has been changed therefore in favor of the current name Systemic sclerosis. Diagnosis and treatment of scleroderma require special medical experience with these diseases.
In recent years, the nomenclature of scleroderma has changed dramatically. Today morphea (localized scleroderma), limited and diffuse skin systemic sclerosis, and systemic sclerosis are accepted forms.
Scleroderma, which also affects the internal organs, also called systemic sclerosis. The so-called CREST syndrome (Calcinosis, Raynaud’s phenomena, Esophageal dysmotility, Sclerodactyly, Telangiectasia) is a sub-form of limited systemic sclerosis and identifies a common pattern of disease. The connective tissue of the lung, the kidney, the esophagus, and the heart is considered to be particularly vulnerable. The pulmonary involvement is now the most common cause of death in systemic sclerosis.
The cause of scleroderma is not known. Genetic factors and pathological autoimmune processes have been demonstrated. There may be stimulatory autoantibodies against the receptor of the growth factor Platelet Derived Growth Factor (PDGF), considered among the causes of the disease. A related cancers was observed. Approximately 2 to 50 in 100 000 people are infected, with at most 50 to 60 cases. Women are about three to four times more frequently affected than men. There are about 1 to 2 cases per 100,000 people / year.
Scleroderma is spreading per se in painless from, but occasionally id associated with the quite painful diseases: arthralgias and myalgias. The rate of disease progression is variable and includes fast curves, gradients over the years and self-limiting forms that come to a stop by itself (common in morphea). Because of the rarity of the disease and the very different course with variable organ involvement, the disease is difficult to diagnose, for example, in the early stages, especially in the absence of typical symptoms.
Early symptoms of generalized scleroderma are the shortening of the lingual frenulum and Raynaud’s syndrome, followied by edema of the hands and feet. The skin becomes rigid to atrophy then. At this stage it looks waxy and thin. Finally hands deform: the fingers remain fixed in flexion (claw hand) and are strongly narrowed (Madonna finger).
Characteristic symptoms in the course are the mask face with rigid facial expression, microstomia (the mouth cannot be opened wide) as well as problems with eyelid closure.
Radially arranged wrinkles around the mouth are called tobacco pouch mouth.
A general “cure” of the disease is currently not possible; therapy is individual to the present symptoms and organ involvement. In patients with pulmonary involvement, for example, a course of the disease may be favorably influenced by cyclophosphamide. The bone marrow transplant is currently being tested. The disease was formerly often treated with glucocorticoids, but we know now that their use in systemic sclerosis is not unproblematic. For example, the increased dose of glucocorticoids leads to the risk of life-threatening renal crisis. Therefore, glucocorticoids do not tend to be used as “basic therapy”.
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