Scleroderma (Dermatosclerosis) : Symptoms, Causes, Diagnosis & Treatment

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Scleroderma (Dermatosclerosis) : Symptoms, Causes, Diagnosis & Treatment

Scleroderma is caused by the immune system attacking the connective tissue under the skin and around internal organs and blood vessels. This causes scarring and thickening of the tissue in these areas. There are two main types of scleroderma, ‘localised’ and ‘systemic’:

Localised – ‘Morphea’ is the name given to localised patches of hardened skin that are smooth and shiny. Usually they appear on the trunk, but they can affect any part of the body. They are painless and there are normally no other problems or symptoms.

In ‘Linear’ scleroderma the skin affected is in a line, usually along an arm or leg. The skin appears shiny, miscoloured or scarred, and often feels tight and uncomfortable. In children, it has to be monitored carefully as the normal growth of limbs can be affected.

Systemic sclerosis – In this type of scleroderma the internal organs are affected as well as the skin. The heart, oesophagus, blood vessels, kidneys, lungs, blood pressure and digestive system can all be involved.

The symptoms of scleroderma vary for each person, and the severity depends greatly on which parts of the body are affected. Usual symptoms include hardening of the skin, swelling of the hands and feet, joint pain and stiffness and blood vessel damage leading to a physical over-reaction to cold or stress, called Raynauds’ Syndrome.

There’s no cure for scleroderma, but most people with the condition can lead a full, productive life. The symptoms of scleroderma can usually be controlled by a range of different treatments.

Scleroderma is more common in women. The morphea type of scleroderma usually strikes people around 20-40 years old. Linear scleroderma often occurs in children. Systemic scleroderma is more likely to occur in people aged 30-50 years old.

It is difficult to self diagnose Scleroderma. Common symptoms include tightening of the skin, joint pain, exaggerated response to cold (Raynaud’s disease) and heartburn

How does Scleroderma affect your body?

Scleroderma is a chronic disease that causes the skin to become thick and hard, a buildup of scar tissue, and damage to internal organs such as the heart and blood vessels, lungs, stomach and kidneys. The effects of scleroderma vary widely and range from minor to life-threatening, depending on how widespread the disease is and which parts of the body are affected.

What are the Causes of Scleroderma?

Scleroderma occurs because part of the immune system becomes overactive and out of control. This leads to cells in the connective tissue producing too much collagen, causing scarring and thickening (fibrosis) of the tissue. It’s not clear why this happens. Certain genes are thought to be involved, and having a close family member with the condition may increase your risk.

What are the Risk Factors of Scleroderma?

Common risk factors include :

Family members with autoimmune disorders

Occupational chemical exposure, such as silica, ketones, or welding fumes

What are the symptoms of Scleroderma?

In case of localized scleroderma, symptoms are usually restricted to the skin. This disorder does not progress to involve internal organs. The skin lesions may completely reverse themselves in a few months or a few years. In some cases, they lead to permanent disfigurement. Symptoms include:

Hard patches on the skin, most often on the face or trunk (morphea)

Lines of thickened skin that can extend to underlying muscles and bones (linear scleroderma or linear morphea)

In case of systemic scleroderma, the disease is typically categorized as either limited or diffuse disease. Many cases of limited disease begin gradually with Raynaud’s phenomenon. This involves swelling, tingling, numbness, blue and white color, and pain in fingers and toes. It is brought on by cold or emotional distress. The condition can progress over the years to thickened skin. Symptoms include :

  • Diffuse thickening and hardening of the skin
  • Joint and muscular pain, stiffness, and swelling
  • Problems with breathing, swallowing, and digesting food due to thickening and hardening of lung, esophagus, bowel tissues
  • Inflammation and thickening of large and small blood vessels

How is Scleroderma diagnosed?

Scleroderma is difficult to diagnose as it overlaps and resembles many other conditions. Depending on your particular symptoms, a diagnosis of scleroderma may be made by a

Physical examination – Finding one or more of the following factors may indicate scleroderma:

  • Changes in skin appearance and texture, including swollen fingers and hands and tight skin around the hands, face, mouth, or elsewhere
  • Calcium deposits developing under the skin
  • Changes in the tiny blood vessels (capillaries) at the base of the fingernails
  • Thickened skin patches.

Lab tests – To help confirm a suspected diagnosis, at least two proteins, called antibodies, are commonly found in the blood of people with scleroderma. These are

Antitopoisomerase-1 or Anti-Scl-70 antibodies which appear in the blood of up to 30 percent of people with diffuse systemic sclerosis and

Anticentromere antibodies which are found in the blood of many people with limited systemic sclerosis.

Skin biopsy – It involves surgical removal of a small sample of skin for microscopic examination to aid in or help confirm a diagnosis. However, skin biopsy results cannot distinguish between localized and systemic disease.

How to prevent & control Scleroderma?

No clinical data is available regarding this.

Treatment of Scleroderma

There is no drug that is proven to stop, or reverse, the symptom of skin thickening and hardening. Symptomatic treatment is given to curb individual symptoms and prevent further complications –

Raynaud’s phenomenon can be treated with drugs such as calcium channel blockers or drugs called PDE-5 inhibitors ⎼ sildenafil (Viagra®), tadalafil (Cialis®) ⎼, which open up narrowed blood vessels and improve circulation.  To prevent further damage, it’s important to keep the whole body warm, especially fingers and toes. It’s also important to protect fingertips and other skin areas from injury, which can happen even during normal daily activities.

Heartburn (acid reflux) can be treated with antacid drugs, especially proton-pump inhibitors (omeprazole and others). These medications ease gastro-esophageal reflux disease (known as GERD).

Scleroderma kidney disease can be treated with blood pressure medications called “angiotensin converting enzyme inhibitors” (ACE inhibitors). These can often effectively control kidney damage if started early and use of these drugs has been a major advance for treating scleroderma.

Muscle pain and weakness can be treated with anti-inflammatory drugs such as glucocorticoids (prednisone), intravenous immunoglobin (IVIg), and/or immunosuppressive medications.  Physical and occupational therapy may be useful to maintain joint and skin flexibility. Early referral to therapy should be considered to help prevent the loss of joint motion and function.

Scleroderma – Lifestyle Tips

Problems with digestion may require changes in diet; patients often have to eat several small meals rather than fewer large meals.

Patients must also keep the skin well-moisturized to lessen stiffness and be careful during activities such as gardening, cooking—even opening envelopes—to avoid finger injuries.

To keep the body warm, patients should dress in layers; wear socks, boots and gloves; and avoid very cold rooms.

Exercise and/or physical therapy may ease stiffness in the joints.

What are Recommended Exercises for Person with Scleroderma?

Since many people with scleroderma experience joint and muscles stiffness, low-impact activities like walking, cycling, swimming, yoga, dancing, water aerobics and pilates are all good choices for activities. However, everyone with sclerodema is different, and so exercise intervention will be very person dependant.

Scleroderma & Pregnancy – Things to know

Women with systemic sclerosis may find it harder to get pregnant and could have a slightly higher risk of miscarriage and giving birth prematurely. However, if the pregnancy is planned in consultation with a doctor during a period when the condition is stable, there’s no reason why a woman with systemic sclerosis cannot have a successful pregnancy.

Common Complications Related to Scleroderma

Common complications include:

  • Bleeding
  • Pulmonary hypertension
  • Heart rhythm problems
  • Heart failure
  • Kidney failure
  • High blood pressure

Other FAQs about Scleroderma

What is the life expectancy of a person with scleroderma?

Women in the group had a life expectancy 22.4 years shorter than the general population. For men, it was 26 years shorter. People with diffuse scleroderma were more likely to die than those with limited disease, with 24.2% of those patients dying within eight years.

Can Scleroderma be reversed?

Researchers say they have discovered a promising target for new drugs that might be able to reverse the fibrosis process in systemic sclerosis or scleroderma – a rare disease that cuts short the lives of many patients and for which there is no cure or effective treatment.

Do scleroderma symptoms come and go?

Scleroderma usually develops in middle age. Scleroderma is a permanent condition, although the symptoms may come and go. Unfortunately, in most cases, the symptoms get worse with time. The exact cause of scleroderma is not known.

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