Scleroderma: causes, symptoms, treatment and prognosis

Scleroderma is not common, it is a condition involving the connective tissue. The tissue becomes inflamed, thickens and damage occurs. Particularly the tissue of the skin, it contracts and hardens. It is most common in people between the ages of 30 and 50. What are the causes and symptoms of scleroderma? How can the condition be treated? Can scleroderma be cured? What does CREST syndrome mean?

Article content

  • Scleroderma
  • Connective tissue
  • Causes of scleroderma
  • Symptoms of scleroderma
  • Two major forms of scleroderma
  • Subdivision of systemic scleroderma: CREST syndrome and diffuse form
  • Diagnosis of scleroderma
  • Scleroderma treatment
  • Scleroderma prognosis

Scleroderma

The name scleroderma comes from the (Greek) word skleros . This means hard and derma means skin. Together they mean hard skin. In scleroderma or systemic sclerosis (SSc), the connective tissue of the skin is thickened or hardened. In addition to the skin, this can also happen to joints and internal organs. Scleroderma occurs all over the world, mainly affecting people between the ages of thirty and fifty. Women are affected by the condition more often than men. Scleroderma is more common in some families and in African Americans. The lifestyle is not a significant factor.

Connective tissue

Connective tissue is fibrous tissue that surrounds other tissues or organs. It also provides support, connects tissues and organs together and holds them in place. Connective tissue is found everywhere in the body, including inside organs. Tendons, membranes, ligaments and scars also consist of connective tissue. There are also benign growths of connective tissue, these are called fibroids. Malignant growths of connective tissue are also known as sarcomas.

Causes of scleroderma

Scleroderma is an autoimmune disease . The body does not recognize its own connective tissue and antibodies are made against the tissue. It is not clear why this happens, but hereditary factors may play a role. This is thought because the disease is common in some families and scleroderma more commonly affects people of African descent.

Symptoms of scleroderma

Scleroderma can affect the skin, joints and internal organs. The symptoms can be mild to severe:

  • Fingers and toes turn white and hurt (Raynaud’s phenomenon, Raynaud’s disease). There is hypersensitivity to cold.
  • The fingers and hands are swollen.
  • Pieces of skin on the fingers can become hardened and ulcers can also develop.
  • Pain in the joints, especially those of the hands.
  • Muscles become weaker.
  • Swallowing can be more difficult if the esophagus has become stiffened.
  • The skin becomes tighter and thicker, especially on the limbs, face and torso.
  • The esophagus is the organ most commonly affected, but it can also affect the lung: pulmonary fibrosis. The lung capacity will decrease as well as the ability to absorb oxygen.
  • Scleroderma can cause high blood pressure, which can ultimately lead to kidney failure.
  • In men, erection disorders may occur, this is because the blood supply to the erectile tissues in the penis is not good. The skin of the penis can become hardened, which can also change its shape.
  • Women may have complaints of dryness in the vagina and vulva. This is because the glands in this part of the body do not work properly. Pain during sex can be the result. In principle, fertility is not reduced in scleroderma.

Two major forms of scleroderma

There are two major forms of scleroderma: local form and systemic form . The local form involves hardening of the connective tissue of the skin and other organs are usually not affected. The systemic form mainly involves hardening of the skin of the hands, feet and face. It is also possible that internal organs will become thicker and this can lead to poorer functioning of these organs.

Subdivision of systemic scleroderma: CREST syndrome and diffuse form

Limited skin form : the skin changes are limited to the forearms, lower legs and face. The limited form is also called CREST syndrome. Symptoms that may occur with this form: deposition of calcium in the connective tissue of muscles and joints, which leads to inflammation of the joints. Raynaud’s disease (see symptoms below), the esophagus may be affected (food does not sink as well), the skin of the fingers may harden (also called sclerodactyly), red spots may be visible on the skin (due to capillaries that dilate).
Diffuse skin form : skin abnormalities on hands, arms, feet, legs and on the trunk and face. Organs can also be affected.

Diagnosis of scleroderma

The doctor will be able to make the diagnosis based on the symptoms and a physical examination. Furthermore, the blood can be checked for certain antibodies. A piece of the skin can also be taken for examination.

Scleroderma treatment

  • Scleroderma cannot be cured .
  • Treatment is aimed at slowing down the process. It is important to reduce damage to organs, and treatment also ensures that symptoms can be alleviated. The thickening of the skin can be slowed down by medication. If someone suffers from Raynaud’s phenomenon (this occurs in ninety percent of people with the condition), vasodilators can be given. It is wise to wear gloves and sturdy socks against the cold. If the lungs are affected, a drug can be used to suppress the immune system. Corticosteroids are an option for joint pain.

Scleroderma prognosis

The course of scleroderma can vary. The condition can have mild symptoms or be serious at first and improve later. In general, the prospects for people with the limited skin form are better than those with the diffuse skin form. With the diffuse skin form, the risk of organ damage to the kidney and/or lungs is much greater. It is very important that a skin score is determined regularly in people with scleroderma, but at least once every six months .