Scleroderma is an autoimmune disease of the connective tissue. Autoimmune diseases are illnesses which occur when the body's tissues are attacked by its own immune system. Scleroderma is an autoimmune disease of the connective tissue. Autoimmune diseases are illnesses which occur when the body's tissues are attacked by its own immune system.

Also called as CREST variant of scleroderma. This name represents the following features:

Calcinosis

• Refers to the formation of tiny deposits of calcium in the skin
•  This is seen as hard whitish areas in the superficial skin, commonly overlying the elbows, knees, or fingers
• These firm deposits can be tender, can become infected, and can fall off spontaneously or require surgical removal

Raynaud’s phenomenon

• Refers to the spasm of the tiny artery vessels supplying blood to the fingers, toes, nose, tongue, or ears.
• These areas turns blue, white, then red after exposure to extremes of cold, or even sometimes with extremes of heat or emotional upset.

Esophagus disease

• In scleroderma is characterized by poorly functioning muscle of the lower two-thirds of the esophagus
• This can lead to an abnormally wide esophagus which allows stomach acid to backflow into the esophagus to cause heartburn, inflammation, and potentially scarring
• This can eventually lead to difficulty in passing food from the mouth through the esophagus into the stomach
• Symptoms of heartburn are treated aggressively in patients with scleroderma in order to prevent injury to the esophagus

Sclerodactyly

• Refers to the localized thickening and tightness of the skin of the fingers or toes.
• This can give them a "shiny" and slightly puffy appearance.
• The tightness can cause severe limitation of motion of the fingers and toes.
• These skin changes generally progress much slower that those of patients with the diffuse form of scleroderma.

Telangiectasias

Are tiny red areas, frequently on the face, hands and in the mouth behind the lips. These areas blanch when they are pressed upon and represent dilated capillaries.

 

Patients can have variations of CREST, for example, CRST, REST, ST, etc.

Patients can also have "overlap" illness with features of both CREST and the diffuse form of scleroderma.

Some patients have overlaps of scleroderma and other connective tissue diseases, such as rheumatoid arthritis, systemic lupus erythematosus, and polymyositis.

When features of scleroderma are present along with features of polymyositis and systemic lupus erythematosus, the condition is referred to as mixed connective tissue disease (MCTD).

Signs and symptoms

• CREST is a limited form associated with antibodies against centromeres and usually spares the lungs and kidneys.
• Patients with CREST commonly and slowly produce a pulmonary artery hypertension which can result in heart failure.
• Blood vessel thrombosis and arteriosclerosis has also led to the necessity of amputation of fingers.
• Open leg sores can result from burst blood vessels and thin skin, leading to chronic infections.
• Other Symptoms of CREST patients can be exhaustion, weakness, and difficulties with breathing, dizziness and badly healing wounds.

Causes

Although an abnormal immune system response and the resulting production of excess collagen appears to be the main cause of limited scleroderma. Other factors include

 

• Genetic make up
• Pregnancy- mothers receive blood and other cells from their fetuses, which pass from a baby to its mother through the placenta, can linger in the mother's body for years after a child's birth.
• Hormones- Estrogen may be a cause
• Environmental factors- exposure to viral infection and toxins can trigger scleroderma

Risk factors

• Age- 30- 65 yrs
• Gender- females are more prone to the disease
• Genetic factors- family history of autoimmune disease
• Exposure to toxins- poly vinyl chloride, benzene, silica

Test and diagnosis

• Skin biopsy
• Anti- centromere antibodies

Complications

• Barrett's esophagus, a serious pre-malignant condition, and to an increased risk of esophageal cancer
• Pulmonary hypertension is a more frequent complication than is interstitial lung disease for people with limited scleroderma
• Digital ulcers- Severe Raynaud's phenomenon can obstruct blood flow to your extremities and may cause ulcers of the fingers and toes
• Arrhythmias and myocarditis
• Arthritis and arthralgia
• Dental problems- microsomia (smaller and narrower mouth), tooth decay, destruction of enamel.
• Depression and anxiety
• Sicca syndrome- sicca syndrome causes dry eyes, a dry mouth and sometimes dry airways.

Treatments for the various aspects of limited scleroderma include:

Calcinosis

• Minocycline can sometimes reduce inflammation and ulcers in severe calcinosis.
• Very large or painful calcium deposits are sometimes surgically removed.
• Carbon dioxide laser may be effective at relieving symptoms and preventing deposits from returning.

Raynaud’s phenomenon

• To keep the body's core temperature elevated and protecting your hands and feet from cold, both indoors and out
• Long acting calcium blockers opens the small blood vessels and improves the circulation
• Topical nitroglycerin, which can help heal skin ulcers

Esophageal dysfunction

• Eating small meals that emphasize soft-textured foods
• Waiting two to three hours after a meal before lying down
• And avoiding alcohol, caffeine, chocolate and spicy foods, which can aggravate heartburn.
• Your doctor may also suggest an H-2 blocker and proton pump inhibitors

Sclerodactyly

• Moisturizers for dryness and cortisone creams for inflammation
• Light therapy (phototherapy) may prove more therapeutic help

Telangiectasias

• Dilated capillaries can be hidden with makeup or reduced or eliminated with laser therapy
• Lung complications- Cyclophosphamide for the treatment of scleroderma-related interstitial lung disease
• Epoprostenol, Bosentan, and Sildenafil are used in treatment of pulmonary hypertension

Morphea

Morphea is scleroderma skin that is localized to a patchy area of the skin that becomes hardened and slightly pigmented.

Sometimes morphea can cause multiple lesions in the skin. Morphea is not associated with disease elsewhere in the body.