Scleroderma

Scleroderma is a clinical condition in which hardening or tightening of the skin and fibrous tissue occurs. In most of the people this disease is only confined to skin but in others it may spread beyond skin- such as blood vessels, digestive tract and internal organs.

Signs and symptoms of the disease may vary depending on which structures are affected.  

Women are more likely to be effected by scleroderma than men and the common age group of this disease is between 30 – 50 years. ]

There is no cure for scleroderma and only symptomatic therapy can be done to improve the quality of life in this disease.

Signs and symptoms of scleroderma may vary depending on the organ involved in this process:

• Skin- common symptom of scleroderma is tightening and hardening. These patches maybe oval shaped of in straight lines. The number location and size vary depending on the type of scleroderma. Appearance of shiny patches and restricted movement will be seen in the disease.
• Fingers and toes-  one of the earliest signs of scleroderma is loss of sensation to cold temperatures which can cause numbness, pain or color changes in the fingers and toes commonly known as Raynaud’s phenomenon.
• Digestive system- in addition to acid reflux which can damage the mucous membranes of the esophagus, some people also notice malabsorption due to decreased intestinal motility.
• Heart, lungs and kidney- rarely scleroderma spreads to these organs and these can cause problems with ventilation in lungs, filtration will be affected in kidneys and in heart rhythm may be disturbed.

Scleroderma is caused by an increased sensitivity of body’s immune system against skin. Due to some unknown sensitivity our body’s own immune cells attack the normal cells of skin and cause their lysis.

Buildup of collagen after the inflammatory response is caused and collagen is a hard protein which gives the characteristic hard and shiny appearance to skin in affected area.

Making diagnosis of scleroderma does not require many tests. But due to different forms of the disease and different location of pathogenesis diagnosis may become difficult. A thorough physical examination will be done.

The doctor will check the tenderness, elasticity and moisture of the skin. Blood tests will be recommended to see the count of red blood cells, white blood cells and allergic mediators. A small biopsy from the affected area will be taken to examine in the laboratory.

In severe cases of scleroderma CT scans from lungs and echocardiogram for heart will be done to access their function.

In some cases, the scleroderma is so mild that it requires no treatment. There are no drugs to treat scleroderma although the symptoms of scleroderma can be decreased to improve the quality of life. Treatments include:

• Dilate blood vessels- blood pressure medications that act by dilating blood vessels may prevent the complications of heart and kidneys.
• Immunosuppressant’s- the major cause is immune cells causing lysis of normal healthy cells, immunosuppressant’s can reduce the scarring.
• Reduce stomach acids- omeprazole will decrease the production of HCL and reduce the damage caused to esophageal mucosa by reflux.  
• Antibiotics- if the immune cells are attacking a pathogen such as bacteria antibiotics can help kill the underlying cause.
• Analgesics- over the counter painkillers can be taken to reduce the symptoms of inflammation.
• Surgery:
  • amputation- in case of severe Raynaud’s phenomenon gas gangrenes are formed which can lead to tissue necrosis and there will be a need of amputation.
  • Organ transplants- if the disease leads to organ failure, organ transplant will save life of the patient.

To prevent scleroderma stress free life can be recommended and healthy diet must be maintained, sometimes the food we eat may cause allergic reactions.

Scleroderma develops due to unknown activation of immune cells against the normal body cells especially skin.

There are no known alternative remedies for scleroderma.  Although, Acupuncture can help relieve symptoms of stress. 

Lifestyle modifications are necessary in order to cope with scleroderma.

Meditation and relaxation will provide peace of mind. And will sped up the process of healing.  

Living with scleroderma will increase the stress levels some of the suggestions which can help the stress are:

• maintenance of normal daily activities,
• pace yourself and stay busy in work,
• social connections with friends will enhance mind,
• continue to pursue hobbies and do what you like to do the most,
• joining a support group to share experiences and understand feelings of the other people suffering from same disease. 

There are several risks and complications associated with scleroderma.

Women are more predisposed than men in scleroderma. Choctaw Native Americans of Europe descent are more likely to develop this disease than Americans of European descent. They develop special type of scleroderma which affects internal organs.

Complications of scleroderma may range from mild to severe and can affect:  

• Fingertips- the Raynaud’s phenomenon can be so severe that it can restrict the blood flow to an extent that it may permanently damage the fingertips causing skin sores, in worst cases gangrene might develop leading to the amputation of the fingertips.
• Lungs – scar tissue (pulmonary fibrosis) will reduce the capacity of breathing, reduce perfusion rate and will decrease the blood flow. In worst cases it may lead to the development of pulmonary hypertension.
• Kidneys- when scleroderma affects kidneys hypertension due to impaired filtration of the blood can develop. Kidney failure will be in extreme cases.
• Teeth- tightening of the skin over face will reduce the opening of mouth and will make it hard to brush teeth leading to dental decay.
• Digestive system- malabsorption due to decreased intestinal motility, constipation and deficiency of essential nutrients to the body will develop.
• Sexual function- scleroderma will decrease erection in males and in females it causes rigidity with less amounts of vaginal fluid is secreted.