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Scleroderma Symptoms 2

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Raynaud’s Phenomenon

Raynaud’s phenomenon is characterized by cold sensations and color changes in the hands and feet. Upon exposure to cold or emotional stress, the fingers and/or toes (sometimes the nose), lose circulation and turn white (blanch). Once the digits are re-warmed the blood flow returns, commonly 10 to 15 minutes later. The affected portion of the digits will often turn a bluish color or will appear mottled before returning to normal appearance.

Several studies have reported that between 4% and 15% of the general adult population, primarily women, have symptoms of Raynaud’s phenomenon. These symptoms are usually quite mild and are not associated with any underlying disease. This form of Raynaud’s is known as “primary Raynaud’s.” It is also associated with a negative ANA. It generally first appears at a much younger age than secondary Raynaud’s, often before the age of 20. When Raynaud’s attacks are intense or long lasting or first occur after the age of 20, there is an increased likelihood that the Raynaud’s is secondary to an underlying autoimmune disorder. Note that in addition to scleroderma, Raynaud’s can be associated with a number of other disorders, for example, lupus, mixed connective tissue disorder, polymyositis, dermatomyositis, cold agglutinin disease, or  hypothyroidism.

With secondary Raynaud’s there will usually be enlargement of the blood vessels at the base of the fingernails (nail bed capillary enlargement), although this is not always the case.

Skin Changes

In the earliest stages of scleroderma, the skin appears mildly inflamed with swelling and often redness. The skin gradually thickens (more rapidly in the diffuse form) and the patient feels progressive “tightening” of the skin with decreased flexibility. The skin changes are more widespread in the diffuse form and the skin can become “hyperpigmented,” giving the skin a salt and pepper appearance.

The pattern of skin changes is different for limited scleroderma and diffuse scleroderma.  With limited scleroderma, the skin changes are typically limited to the fingers and lower arms, toes and lower legs, and the face.  With diffuse scleroderma, the changes can cover more of the body including upper arms and legs and the trunk area.

As the skin changes progress, the skin becomes thicker and can become severely dried with intense itching. This stage can progress for a long period, up to several years. With diffuse scleroderma, the inflammation and further thickening stops as the skin begins to thin, although the skin will usually bind with underlying structures. Painful ulcerations can occur at joints.

With the limited form of the disease calcium deposits may form under the skin. These can appear as white spots or ulcerations and may be quite painful. Spider veins (telangiectasia) often appear on the fingers, chest, face, lips, and tongue.

Musculoskeletal (Muscles and Joints)

Nonspecific muscle pain and stiffness are often some of the earliest symptoms of scleroderma. While arthritis can also occur, the pain and stiffness over the joints is greater than would normally be expected based on the degree of inflammation visible. Pain can also occur along tendons and into muscles of the arms and legs. This can occur with movement of the ankles, wrists, knees, or elbows. These symptoms are more common in the diffuse form of the disease.

Often, a grating sound can be heard as the inflamed tissues move over each other, particularly at and below the knees. With diffuse scleroderma, the fingers, wrists, and elbows can become fixed in flexed positions because of the scarring of the skin. In the limited form, this is usually limited to the fingers.

In later stages of the disease, muscle loss and weakness are the main problems. In some cases, however, some of the symptoms may be caused by some of the drugs commonly used to treat scleroderma, such as steroids.

Recent research has isolated which subtypes of Scleroderma are more likely to have muscle problems.

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