plz im in need n help mee.....
thnx I had a form of scleroderma...called Morphea.
It is found usually along one side of the body, and also tends to run it's course, as opposed to other forms of scleroderma, which progressively invade all organ systems, starting with the integumentary system (skin).
I also had this at a very young age (age 3-9 from start to finish). My doc was a dermatologist. Dr. Mitchell Ede in Cincinnati OH. His daughter has taken over his practice since his retirement.
I also visited the Mayo Clinic in Rochester MN on several occasions. It was very rare for a child to have morphea, so I was quite the spectacle.
He was the only doc in our entire area who knew ANYTHING. He put me on high doses of corticosteroids and vitamin E.
Best of luck to you. I hope I may have helped in some way. There is no cure for every patient with scleroderma, though *there is treatment* for some of the symptoms, including drugs that soften the skin and reduce inflammation. Some patients may benefit from exposure to heat.[citation needed]
A range of NSAIDs (nonsteroidal anti-inflammatory drugs) can be used to ease symptoms, such as naproxen. If there is esophageal dysmotility (in CREST or systemic sclerosis), care must be taken with NSAIDs as they are gastric irritants, and so a proton pump inhibitor (PPI) such as omeprazole can be given in conjunction.
Immunosuppressant drugs, such as mycophenolate mofetil (Cellcept庐) or cyclophosphamide are sometimes used to slow the progress.
Digital ulcerations can be helped by prostacyclin (iloprost) infusion. Iloprost being a drug which increases blood flow by relaxing the arterial wall.
Causes
There is no clear obvious cause for scleroderma and systemic sclerosis. Genetic predisposition appears to be limited: genetic concordance is small; still, there often is a familial predisposition for autoimmune disease. Polymorphisms in COL1A and TGF-尾1 may influence severity and development of the disease. There is limited evidence implicating cytomegalovirus (CMV) as the original epitope of the immune reaction, and organic solvents and other chemical agents have been linked with scleroderma.[16]
One of the suspected mechanisms behind the autoimmune phenomenon is the existence of microchimerism, i.e. fetal cells circulating in maternal blood, triggering an immune reaction to what is perceived as "foreign" material[19].[16]
Pathophysiology
The overproduction of collagen is thought to result from an autoimmune dysfunction, in which the immune system would start to attack the kinetochore of the chromosomes. This would lead to genetic malfunction of nearby genes. T cells accumulate in the skin; these are thought to secrete cytokines and other proteins that stimulate collagen deposition. Stimulation of the fibroblast, in particular, seems to be crucial to the disease process, and studies have converged on the potential factors that produce this effect.[16]
A significant player in the process is transforming growth factor (TGF尾). This protein appears to be overproduced, and the fibroblast (possibly in response to other stimuli) also overexpresses the receptor for this mediator. An intracellular pathway (consisting of SMAD2/SMAD3, SMAD4 and the inhibitor SMAD7) is responsible for the secondary messenger system that induces transcription of the proteins and enzymes responsible for collagen deposition. Sp1 is a transcription factor most closely studied in this context. Apart from TGF尾, connective tissue growth factor (CTGF) has a possible role.[16]
Damage to endothelium is an early abnormality in the development of scleroderma, and this too seems to be due to collagen accumulation by fibroblasts, although direct alterations by cytokines, platelet adhesion and a type II hypersensitivity reaction have similarly been implicated. Increased endothelin and decreased vasodilation has been documented.[16]
Jimenez & Derk[16] describe three theories about the development of scleroderma:
The abnormalities are primarily due to a physical agent, and all other changes are secondary or reactive to this direct insult.
The initial event is fetomaternal cell transfer causing microchimerism, with a second summative cause (e.g. environmental) leading to the actual development of the disease.
Physical causes lead to phenotypic alterations in susceptible cells (e.g. due to genetic makeup), which then effectuate DNA changes which alter the cell's behavior. |
