Sclerotherapy

Scleroderma

Manifestation of scleroderma on the skin. Image courtesy of Wiki Commons.

Scleroderma is an autoimmune disease characterized by hardening and tightening of the skin and connective tissues. While the most evident manifestations are on the skin, scleroderma can affect various internal organs, blood vessels, and the digestive tract, leading to diverse complications.

Overview

The term "scleroderma" originates from the Greek words "skleros" (hard) and "derma" (skin). It aptly describes the skin's appearance and texture in affected individuals. The disease's exact cause remains unknown, but it involves an overproduction of collagen, leading to the skin and organ changes observed.

Classification

Scleroderma is broadly categorized into:

• Localized Scleroderma: Affects only the skin and, in some cases, the muscles underneath. It does not impact internal organs. Examples include:

 * Morphea
 * Linear scleroderma

• Systemic Scleroderma (or Systemic Sclerosis): Affects the skin as well as internal organs. It is further divided into:

 * Limited cutaneous systemic sclerosis
 * Diffuse cutaneous systemic sclerosis

Causes

The precise cause of scleroderma is not well-understood, but it is believed to be an autoimmune disease. Factors include:

• Genetics: Family history can sometimes play a role.
• Environmental triggers: Certain toxins or infections may trigger the onset in genetically predisposed individuals.
• Vascular abnormalities: Blood vessel abnormalities can lead to tissue damage and additional symptoms.

Symptoms

The symptoms of scleroderma vary based on the type and the areas of the body affected. Common symptoms include:

• Hardened or tightened patches of skin
• Raynaud's phenomenon, where fingers or toes turn blue or white in response to cold temperatures
• Acid reflux or heartburn
• Swollen or painful joints
• Dry eyes and mouth
• Shortness of breath or lung complications
• Kidney problems

Diagnosis

Scleroderma can be challenging to diagnose due to its varied manifestations. Diagnosis usually involves:

• Clinical Examination: Observing the changes in the skin and nails.
• Blood tests: Looking for specific antibodies common in scleroderma.
• Skin biopsy: Examining a small skin sample under a microscope.
• Organ-function tests: To check the extent of internal organ involvement.

Treatment

While there is no cure for scleroderma, treatments aim to manage symptoms and prevent complications. Approaches include:

• Medications: To address specific symptoms like heartburn, joint pain, or blood pressure.
• Physical therapy: To maintain mobility of the joints and skin.
• Surgery: In advanced cases, such as to amputate affected fingertips with severe Raynaud's phenomenon.

Prognosis

The prognosis for localized scleroderma is generally good, though changes to the skin can be permanent. Systemic scleroderma, particularly when vital organs are affected, can pose severe health challenges.

References

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