Scleroderma - an auto immune disease that affects the skin

Scleroderma is an auto immune disease that affects the skin. This affect connective tissues leading to inflation, scars and thickening of skin. Most commonly skin over tips of elbows, fingers and face. Mostly women are more affected by the disease than men. Scleroderma can be classified into two diffuse and limited. In diffuse form skin of face, chest, abdomen etc thickens symmetrically. Some times organ diseases also occur which might be serious. Bowels, lungs, heart, esophagus and kidneys are most commonly affected. It can even lead to high blood pressure. In limited form only skin of face and fingers are effected. This effect takes place slowly and is also known as CREST. As per this name CREST

C stands for tiny calcium deposits called calcinosis, commonly found in elbows, knees etc in white colour in superficial skin. This might need a surgery or might be tender enough to fall off if infected.

R is for Raynaud's phenomenon which is spasm of small artery vessels. Commonly they are found in arteries to fingers, nose, tounge, ears or toes. They change colour to blue, white and then red when exposed to extreme heat, cold or emotions.

E goes for Esophagus, the condition in which muscles function poorly or lower part of esophagus stops functioning. Esophagus may widen leading to backflow of stomach acid causing heartburn, scarring or inflammation.

S refers to Sclerodactyly ie. tightening or thickening of skin of toes and fingers. This may limit the motion of fingers and toes and they will appear puffy and shiny.

Finally T is for Telangiectasias, tiny red spots that blanch on pressing and represents dilated capillaries on face, mouth or hands. It is not necessary that all these symptoms exist along.

When the disease diffuse it is called as systemic sclerosis. Some might also be affected with some other connective tissue diseases. When symptoms of scleroderma are found with systemic lupus erythematosus symptoms or of polymyositis it is called mixed connective tissue disease (MCTD). Linear scleroderma is a condition in which hardened skin is found as a strip along leg. If affected to a child this can stunt bone growth of affected leg. The disease can be dangerous to your heart leading to conditions like myocardial disease, arrhythmias or conduction system abnormalities. Kidneys and lungs are also affected due to tightening of blood vessels.

Symptoms

Irritation
Skin hardening
Pigmentation
Inflammation
Progressive tissue fibrosis
Occlusion of the microvasculature
Calcium gets deposited in connective tissues
Blood vessels of hands and feet narrows
Swellimg of esophagus
Red spots on face and hands
Hypertension
Sensitivity to cold

Causes

It is found that genes has a role in causing Scleroderma. Cellular and humoral immunological derangement is considered as a cause of this disease. Activation of fibroblast and injury to Endothelial cell are other causes. At times environment also plays a vital role. Exposure to silica, to solvents like trichloroethylene, benzene, vinyl chloride, epoxy resins, carbon tetrachloride, radiation or radio therapy are environmental factors leading to this disease. When environmental or genetic factors activate the immune system causing injury to tissues patient is affected by this disease. parvovirus B19 is an accelerating agent like human herpesvirus 5 and Cytomegalovirus. Families with Scleroderma patients are found to be affected with other autoimmune diseases also. Body tissues are affected by its own immune system leading to this disease. Certain drugs like Bleomycin and pentazocine can also cause this disease.

Treatment

As this particular disease can lead to several other problems it is better to consult a rheumatologist. He will refer you to other specialists like a dermatologist, a nephrologist, a cardiologist, a gastroenterologist, a pulmonary specialist etc for different problems faced by you. Medicines like Pantoprazole (Protonix), is used to treat ulcer in stomach. For that affecting heat medicines like nifedipine, bosentan, nicardipine are prescriped as they are calcium channel blockers. Skin discolouration and hardening is treated with corticosteroids or moisturizers. In extensive form antibiotics like minocycline is prescribed. Phototherapy also gives some positive results. Vasodilators are used for relaxation and there by help to expansion of blood vessels. Angiotensin converting enzyme (ACE) inhibitors can be given to maintain blod pressure. Aspirin, naproxen, ibuprofen etc can be given as Nonsteroidal anti-inflammatory drugs. Antacids help digestion and decrease acidity while D-penicillamine delays the spreading of disease. Steroids are prescribed to reduce inflammation. Cyclophosphamide and methotrexate are immunosuppressants. At times cosmetic or orthopedic surgery is necessary to correct contractures of joints, to treat scars or skin deformities. Stretching exercise helps to keep joints flexible and strong. Avoiding Vitamin C greater than 100mg/day and excessive smoking are other methods necessary.

Alternative medicine

It is found that herbs can cure scleroderma to a good extent. Extracts from hydrangea root, a herb commonly used in Chinese medicine was proved effective in halting the progress of an autoimmune disease in lab and is yet to be proved on a human body. Acupuncture can relieve from the symptoms of sclerosis. Among Chinese medications are treatments using medical extracts from dried beetles, earth worms, scorpions, centipedes etc. One of the chinese medicines are Yangshen Huoxue Tang a combination of extracts to warm kidney and vitalize blood. Different herbs used in Chinese medicine are effective blood vitalers, yang tonics, spleen qi tonics and warming agents. Rehmania is a common herb used in medicines. Cornus is another herb used to treat kidney. Meditation and yoga are found effective to reduce tension, depression and anxiety caused by scleroderma. Transcendental Meditation is helpful to reduce brain's response to pain. It is said that you can control the disease by changing life style, by introducing healthy diet, regular physical exercise and body cleansing along with different natural traditional therapies.


Links

http://emedicine.medscape.com/article/331864-treatment

http://scleroderma.jhmi.edu/patients/treatment-options.html

http://www.healthscout.com/ency/68/311/main.html

http://arthritis.about.com/od/sclero/Scleroderma_Cause_Diagnosis_Symptoms_Treatment.htm

Achalasia - difficulty in swallowing

Achalasia is a disorder of the esophagus (the tube that carries food from the mouth to the stomach). The esophagus is less able to move food toward the stomach, and the muscle from the esophagus to the stomach does not relax as much as it needs to during swallowing. It is also known as esophageal achalasia, achalasia cardiae, cardiospasm,dyssynergia esopgagus and esophageal aperistalsis. Achalasia is characterized by difficulty in swallowing, regurgitation and some times chest pain.

Symptoms:

Difficulty in swallowing (Dysphagia) liquids and solids, Regurgitation(backflow) of food, coughing, chest pain, hurtburn, weight loss etc are symptoms.

Causes:

There is no known underlying cause of this disease.It may be caused by failure of the lower esophageal sphincter to relax and an absence of esophageal peistalsis leading to a functional obstruction at the gastro-esophageal junction. Esophageal cancer or chagas disease (an infectious disease common in South America) caused achalasia. Another part of the disorder is lack of nervous stimulation to the esophagus. Causes include damage to the nerves to the esophagus, parasitic infection, cancers, and hereditary factors.

Treatment:

Several options are available for the treatment of achalasia. Treatments include: Drug Therapy and botulinum toxin injection, Balloon dilation and surgery (myotomy), Esophagectomy. Injection of botulinum toxin into the lower esophageal sphincter is an effective, safe, and simple method of treatment for achalasia. Medications such as long-acting nitrates such as isosorbide dinitrate or calcium channel blockers such a nifedipine and nitroglycerin can also be used to lower the pressure at the lower esophagus sphincter. Pneumatic dilation is most effective on the long term in patients over the age of 40. Because of excellent results, a short hospital stay, and a fast recovery time, the primary treatment is considered by many to be a laparoscopic Heller myotomy and partial fundoplication surgery (called an esophagomyotomy) to decrease the pressure in the lower sphincter may be needed. A laparoscopic Heller myotomy is considered by many to be the appropriate primary treatment of patients with achalasia .The thoracic or thoracoscopic approach may be preferred for some patients. The thoracic approach is also appropriate in managing patients with proximal esophageal motility abnormalities.

Prevention:

Avoid foods like ketchup,citrus,chocolate,mint,alcohol and cafeein etc.Add dietary food in the food menu.Eat slowly,chew very well,drink plenty of water with meals and avoid eating near bed time.

Alternative Treatment:

Acupuncture,traditional chinese herbal medicine and relaxation techniques are different altrnative method. Raising the head of the bed or sleeping with a wedge pillow promotes emptying of the esophagus by gravity. Proton pump inhibitors can help to prevent reflex damage by inhibiting gastric acid secretion.

Links:

http://content.nejm.org/

http://www.ncbi.nlm.nih.gov/

http://linkinghub.elsevier.com/

http://en.wikipedia.org

http://www.uptodate.com/

http://www.nlm.nih.gov/

http://medind.nic.in

http://www.emedicine.com/

http://www.ssat.com/