What is Scleroderma?

SCLERODERMA

Scleroderma is an uncommon disease of the immune system, blood vessels and connective tissue. In this condition the skin, usually of the hands and feet, becomes stiff, tight and shiny. As a result of changes in these three areas, the body produces too much collagen. Collagen which is the major protein of the connective tissue, binds the body together and is found in the skin, blood vessels, joints and internal organs, such as the kidneys, heart, lungs and bowel. There are several types of collagen and different forms are found in different parts of the body. They are produced by cells called fibroblasts. When fibroblasts produce too much collagen it results in fibrosis, i.e. thickening.

In addition to affecting the fingers, the fibrosis may spread to other areas and organs of the body. The arms, face, trunk and legs may be involved and movement of the limbs may become limited.

Damage to the blood vessels is usually seen in the form of severe Raynaud's, painful pits or scars on the finger tips, telangiectasia and ulcers. These are external signs of scleroderma but internal vascular damage may also occur.

The immune system in a human is composed of specialised cells (lymphocytes, monocytes). Their role is to ward off foreign substances by recognising that they are foreign and mounting an attack, whether it be a bacteria or virus, a chemical agent or a transplanted organ. When the immune system responds to and destroys substances that are not foreign but in fact healthy tissues, the result is an immune injury. In scleroderma this injury probably starts a process which becomes persistent and can be severe.

Symptoms

Some types of scleroderma affect only the skin, while others affect the whole body.

• Localized scleroderma usually affects only the skin on the hands and face. It develops slowly, and rarely, if ever, spreads throughout the body or causes serious complications.
• Systemic scleroderma, or sclerosis, may affect large areas of skin and organs such as the heart, lungs, or kidneys. There are two main types of systemic scleroderma: Limited disease (CREST syndrome) and diffuse disease.

Skin symptoms of scleroderma may include:

• Fingers or toes that turn blue or white in response to hot and cold temperatures (See: Raynaud's phenomenon)
• Hair loss
• Skin hardness
• Skin that is abnormally dark or light
• Skin thickening, stiffness, and tightness of fingers, hands, and forearm
• Small white lumps beneath the skin, sometimes oozing a white substance that looks like toothpaste
• Sores (ulcers) on the fingertips or toes
• Tight and mask-like skin on the face

Bone and muscle symptoms may include:

• Joint pain
• Numbness and pain in the feet
• Pain, stiffness, and swelling of fingers and joints
• Wrist pain

Breathing problems may result from scarring in the lungs and can include:

• Dry cough
• Shortness of breath
• Wheezing

Digestive tract problems may include:

• Bloating after meals
• Constipation
• Diarrhea
• Difficulty swallowing
• Esophageal reflux or heartburn
• Problems controlling stools (fecal incontinence)

Signs and tests

The health care provider will perform a physical exam. The exam may show hard, tight, thick skin.
Your blood pressure will be checked. Scleroderma can cause severe inflammation of small blood vessels, such as those in the kidneys. Problems with your kidneys can lead to high blood pressure.
Blood tests may include:

• Antinuclear antibody (ANA) panel
• Antibody testing
• ESR (sed rate)
• Rheumatoid factor

Other tests may include:

• Chest x-ray
• CT scan of the lungs
• Echocardiogram
• Urinalysis
• Tests to see how well your lungs and gastrointestinal (GI) tract are working
• Skin biopsy

Treatment

 

There is no specific treatment for scleroderma.
There Is No Cure 
Your doctor will prescribe medicines and other treatments to control your symptoms and prevent complications.
Medicines used to treat scleroderma include:

• Power anti-inflammatory medicines called corticosteroids
• Immune-suppressing medications such as methotrexate and Cytoxan
• Nonsteroidal anti-inflammatory drugs (NSAIDs)

Other treatments for specific symptoms may include:

• Medicines for heartburn or swallowing problems
• Blood pressure medications (particularly ACE inhibitors) for high blood pressure or kidney problems
• Light therapy to relieve skin thickening
• Medicines to improve breathing
• Medications to treat Raynaud's phenomenon

Treatment usually also involves physical therapy.

I have used information from: PubMed Health and RSA