What you need to know about Scleroderma

Scleroderma is a chronic autoimmune disease of the connective tissue characterized by fibrosis (hardness), thickening, self-generated scarring, vascular alterations, and inflammation. Autoimmune diseases like scleroderma happens when our own immune system attacks the body's tissues. In the case of scleroderma, fibrosis and thickening of the skin and other body parts happen.

Causes
Scleroderma is sometimes mistaken as a skin condition because most symptoms first affect the skin. Until now, there is no known cause of this condition. Scleroderma may be inherited but studies have not yet identified any gene defect in patients with scleroderma. The process of the disease happens when endothelial cells of small blood vessels and smooth muscle cells die off and get replaced with collagen and other fibrous material.

Major Types
There are two major forms of scleroderma namely, limited systemic scleroderma and diffuse systemic scleroderma.

• Limited Systemic Scleroderma- This mainly affects the skin of the hand, face and arms. Manifestations of this form are of the cutaneous type. The skin changes and other features of scleroderma diffuse slowly in this type of disease. It is also known as the CREST syndrome. CREST represents:

- Calcinosis
- Raynaud's phenomenon
- Esophageal dysfunction
- Sclerodactyly
- Telangiectasias

Pulmonary hypertension can also occur with patients having limited systemic scleroderma and is considered the most dangerous complication of this type.

• Diffuse Systemic Scleroderma - This disabling type diffuses rapidly and affects the large area of the skin and one or more internal organs. This disease frequently affects the esophagus, kidney, heart and lungs.

Systemic Sine Scleroderma - Minor Type
In addition to the two major types, another type of scleroderma is also classified and it is called systemic sine scleroderma. This minor type of the disease is characterized by lack of skin changes, systemic manifestation, morphea, and linear scleroderma. Morphea is defined as a localized patchy area of the skin that becomes hardened and slightly pigmented. On the other hand, linear scleroderma is defined as a strip of hardened skin usually affecting the lower extremities.

Symptoms and Complications
Patients with scleroderma can also acquire connective tissue complications such as rheumatoid arthritis, systemic lupus erythomatosus, and polymyositis. When features of scleroderma are present along with lupus and polymyositis, this disease is called mixed connective tissue disease.

Cutaneous manifestations of the disease include local or widespread redness, swelling, itching, and pain that lead to skin tightening and hardening.

Raynaud's phenomenon can also happen involving the tips of the fingers and toes that causes tiny ulcerations and dead skin.

Internal organs that are involved may result in scarring, increase in blood pressure, fatigue, headache, difficulty of breathing, constipation, diarrhea, and cramping.

Deaths mostly occur because of kidney, pulmonary and heart complications.

Treatments
There is no direct treatment for scleroderma because the nature of the disease is idiopathic (of unknown cause). Treatment for this disease is patient-specific and will depend on the symptoms an individual is manifesting.

For patients experiencing Raynaud's phenomenon, medications such as nifedipine, amlodipine, diltiazem, felodipine and nicardipine are given to increase blood flow to fingers and toes.

Fibrosis of the skin can be treated with agents such as D-penicillamine, colchicine, relaxin, and cyclosporine.

Moreover, immunosuppressive agents are given to halt the progress of autoimmune process. Drugs such as methotrexate, cyclophosphamide, azathioprine, and mycophenolate are used to suppress the immune system.

Anti hypertensive drugs are also given to lower blood pressure caused by internal organ complications.

 


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