Scleroderma: Symptoms, Types, Diagnosis and Treatment

by | Jul 3, 2019 |

Scleroderma is also known as systemic sclerosis. It is a chronic autoimmune rheumatic disease of connective tissues. In this disease, a protein, collagen, is formed in the excessive amount in the extracellular spaces between the connective tissues. This results in the thickening and tightening of the skin. Therefore, the most common sign of the disease is the hardening of the skin. Blood vessels may become thickened and function improperly that further leads to high blood pressure and tissue damage. Scar formation on the lungs and kidneys can also be observed.

Symptoms

Many body parts can be affected by scleroderma with the appearance of the following symptoms:

  • Thickened or hardened or skin with a shiny and smooth look. It is most common on the face and hands
  • Narrowing of the hands and feet’s blood vessels resulting in cold toes or fingers that turn red, blue, or, white (Raynaud’s phenomenon)
  • Sores or ulcers or on fingertips
  • Red small spots on the chest and face (open blood vessels known as telangiectasia)
  • Swollen or puffy or painful toes and/or fingers
  • Swollen and/or painful joints
  • Weakness of muscles
  • Dry mouth or eyes (Sjogren’s syndrome)
  • Shortness of breath
  • Weight loss, heartburn, and diarrhea

Types of Scleroderma

Scleroderma has been divided into two types.

Localized scleroderma: It generally affects skin. It is subdivided into two types, ie, morphea and linear.

  • Morphea: It causes oval-shaped, hard, red or purple patches on the skin with a whitish center. This type of scleroderma may also affect internal organs and/or blood vessels.
  • Linear: It causes lines or streaks on thickened skin of arms, legs, or face.

Systemic scleroderma: It is also known with alternative name as generalized scleroderma, and it may affect many parts of the body. It is subdivided into two types, ie, Limited scleroderma and diffuse scleroderma.

  • Limited cutaneous systemic sclerosis syndrome (CREST): This is the least severe type and affects the face, hands, feet, and lower legs and arms. It may affect the lungs, blood vessels, and the digestive system. It is also known as CREST as the symptoms form the acronym CREST, ie,
    • C: Calcinosis (calcium deposits under the skin and in tissues)
    • R: Raynaud’s disease
    • E: Esophageal complications
    • S: Sclerodactyly (thick skin) on the fingers
    • T: Telangiectasias (enlarged blood vessels) characterized by red spots
  • Diffuse scleroderma: In this, the skin thickening commonly affects hands to above the wrists area and the internal organs. It may cause fatigue, weakness, difficulty swallowing and breathing, and weight loss.

Diagnosis

A patient suffering from scleroderma is asked for health history and is advised for:

  • X-ray
  • blood tests
  • skin biopsy
  • Heart, lungs, and esophagus examinations.

Treatment of Scleroderma

Currently, there is no treatment to inhibit the overproduction of collagen. Only organ system problems can be treated to improve organ functionality and minimize any damage.

  • NSAIDs (nonsteroidal anti-inflammatory drugs) including aspirin, ibuprofen, etc. are used to reduce swelling and pain
  • Immunosuppressants are used to control the immune system to treat problems of joints, muscles or internal organs
  • Blood pressure medications are used to dilate the blood vessels which may help in the prevention of tissue scarring.
  • Some drugs are used to improve blood flow to the fingers
  • Laser surgery and ultraviolet light therapy may also be helpful in improving the appearance and the condition of the skin.

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