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Scleroderma

What is Scleroderma ?

Scleroderma is described as the hardening or thickening of the skin and connective tissues because of the buildup of collagen in the body tissues. It may either solely affect the skin or all parts of body including the internal organs. It can produce mild to severe symptoms. People affected by this condition will notice patches of thickened skin on their fingers and the hands appear swollen. Also, they may notice some skin discoloration and numbness in their extremities following cold exposure or stress.

Scleroderma may not be contagious or cancerous but it can have a huge impact on the daily functioning and self-esteem of the individual. The treatment aims to help deal with the symptoms and prevent serious complications. It is designed based on the patient’s needs after a thorough medical evaluation involving blood works, tissue sampling, skin assessment and other specialized diagnostic tests. Treatment may involve medications, therapies and surgery.

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Causes of Scleroderma

Scleroderma occurs when the body produces too much collagen causing it to gather or accumulate in the body tissues. Collagen, a fibrous protein, is embedded in the connective tissues to provide shape and support making them firm and strong. It is also found in the skin to make it flexible and resilient. Experts do not exactly know what prompts the body produce too much collagen. However, they have identified possible triggers, like:

  • Abnormal immune attack

This abnormal thickening and hardening of the skin has been tagged as an autoimmune disorder. This abnormal immune response attacks and damages the area where collagen is produced and found, such as the dermis, blood vessels and internal organs.

  • Genes

Experts believe that some genes are involved in causing excessive collagen production based on the rate of occurrence of this problem in families with a history of rheumatic disorder. Even so, they have yet to identify the specific genes.

  • Environmental stimulants

Exposure to industrial solvents and silica dust has been thought to trigger scleroderma as well. This also covers the chemicals used during chemotherapy.

Anyone can develop scleroderma but research shows that women, specifically in their 30s and 50s are primarily affected, being responsible for approximately 80% of cases in the US alone.

Appearance of Scleroderma

Each person exhibits the condition differently because it comes in different forms. Scleroderma can be localized or systemic. Other forms of this condition are sub-classifications of the two major forms.

  • Localized

This is a mild form of the disorder affecting only the skin, and mostly, children. Localized scleroderma can be morphea or linear. The morphea type is manifested as thick waxy patches on the skin surface, while the linear type is exhibited as streaks of thick and waxy patches on the forehead, arms or legs.

  • Systemic

This is the severe form of scleroderma wherein the internal organs are affected, and mostly occurs in adults. It can be limited or diffuse. Thickening of connective tissues are less severe and less widespread in limited systemic which develops slowly. This is in contrast to diffuse systemic which develops more quickly and affects more areas of the skin and internal organs.

Scleroderma is initially manifested as a bluish discoloration of extremities following stress or cold exposure. Swollen fingers, hands and feet as well as thick skin patches are also evident. Additional symptoms appear when excessive collagen affects the internal organs. This however depends on the organ/s being affected.

Diagnosis

The patient should expect to undergo a series of tests which will help the doctor make a definitive diagnosis. He or she may ask assistance from a skin specialist and a rheumatologist to figure out the extent of damage and better classify the condition. Diagnosis may involve blood works, skin assessment and getting a tissue sample. Other tests may be required to determine the extent of damage on affected internal organs.

No matter how mild the condition may be, there is a possibility for it to aggravate into a more serious condition. That is why timely diagnosis and treatment is essential in reducing the chances of irreversible damage.

Treatment

Experts have not yet found the solution to restore normal collagen production. The current treatment regimen is more of a supportive care to help the patient manage the condition and prevent serious complications. It is individually designed as each person experiences symptoms at varying degrees.

Appropriate medications are prescribed to treat specific conditions. For instance, medications to increase blood flow to the extremities are often used to treat Raynaud’s phenomenon or the bluish discoloration of the extremities.  Meanwhile, NSAIDs and corticosteroids help relieve esophageal irritation and heartburn, whereas topical creams and lotions can aid with the skin texture.

Therapies can be also helpful in maintaining physical flexibility and other related problems. In some cases, surgery might be needed to resolve the accompanying problems. Patients should not try to treat scleroderma on their own. The condition requires proper evaluation to determine its type, extent of damage and severity.

Scleroderma Pictures

 

 

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