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Scleroderma

What is it?

It is an autoimmune disease (the immune system attacks its own body structures) in which there is hardening of the skin, which becomes thickened, smooth and inelastic.

The name scleroderma derives from from Greek, meaning "hard skin." There are varieties that affect only the skin and a form that affects the skin, blood vessels and internal organs.

Characteristics and symptoms

Scleroderma can manifest itself in many forms. The types that present only skin alterations are named according to the format and extent of the lesions. The disease form areas of hard and smooth skin, hairless and of ivory aspect, mainly located in the trunk, upper limbs and lower limbs.

Scleroderma may manifest in the following ways:

  • drops: the lesions are small, with the size of drops.
  • plaques: the lesions are larger, rounded or oval.
  • linear: the lesions are elongated and unilateral. Linear scleroderma that occurs on the forehead and scalp is known as scleroderma "in coup de saber" due to the historical description of the similarity with the scar of a wound made by a sword.
  • segmented: it is also unilateral, but larger areas are affected. The skin is attached to deep structures, with involvement of the muscles, causing deformity in the affected region, which may be the face, arms or legs.
  • generalized: several lesions widespread affecting large extention of skin.

The type that affects the skin and internal organs is known as progressive systemic sclerosis. Besides the diffuse hardening of skin, alterations occur in blood vessels, muscles, joints, gastro-intestinal tube, heart, lungs and kidneys. The progressive hardening of the skin can lead to difficulties in performing simple movements, such as closing the hands or bending the arms.

One clinical manifestation of this form is quite characteristic, a purplish discoloration that occurs on the fingers and toes, due to decreased blood supply, known as Raynaud's phenomenon.

Treatment

The treatment of forms restricted to the skin is accomplished with the application of drugs by local massage or infiltrations. In forms that have deformities, physiotherapy is important to prevent the impairment of movements. In the generalized forms, oral medications are needed.

Progressive systemic sclerosis is usually treated by a rheumatologist or general practitioner due to the involvement of internal organs. Drugs used for its control may cause significant side effects.

To prevent Raynaud's phenomenon, the extremities should be kept warm with the use of gloves and socks and the avoidance of contact with cold water.

Related articles

- Lupus
- Liche sclerosus


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