Your body has produced extra collagen in your skin. Collagen is what makes your skin firm and also what your body makes to help heal wounds. In Scleroderma, the cells start making collagen as if there were an injury that needs to be fixed. The excess collagen gets into the dermis layer of the skin and causes it to thicken and feel tight and hard.
The degree of skin involvement varies from patient to patient a great deal. Most have “limited scleroderma” with only the fingers and mild face involvement. Others have “diffuse scleroderma” with many areas of the body affected. Skin thickening usually begins on the fingers. In the diffuse form it can, over time, be on the back of the hands, the forearms, the upper arms, the face, the neck, the trunk, the legs or the feet. Similar to thickening of the skin, fibrosis or excess collagen can even occur in the internal organs.
In general, without treatment, the skin thickening will reach its peak within 1-2 years after it starts and will then begin to loosen. How much it will loosen will vary from person to person.
Some areas may get darker (hyperpigmentation) while other areas may develop a patchy loss of skin color (hypopigmentation). Large patches of pigment loss can also happen. The thickened skin can look shiny and scaly from being dry. The pigment changes get better over time and the skin tone usually returns to its normal appearance after several years. You may also develop red spots caused by the widening of small blood vessels in the surface of the skin. These changes are not painful but may be a cosmetic problem.
Generally it is best not to try to change the color with drugs or creams but heavy cosmetics that cover better are the most helpful. Avoidance of sun will prevent increased pigment and stopping any scratching of the skin will reduce damage and prevent loss of pigment. Creams and lotions that moisten the skin can prevent further skin damage. Prescription creams with vitamin A are sometimes given.
The most common visible change is tight skin on the fingers causing bending of the fingertips toward the palm. Tight skin on the face may reduce the size of a person’s mouth opening, create a few small vertical lines around the lips and in some cases interfere with drawing the lips over the teeth. You may experience hair loss on the arms and legs, sweat less or develop skin sores where the skin is injured by trauma. Some patients will develop small calcium deposits in the tissues of the fingers, in the bursa (sac) over the tip of the elbows, rarely over the kneecaps or in other pressure areas. If the deposits open, they may become infected. You should contact your doctor if this happens.
Dry skin is caused by several things. First the extra layer of collagen in the inner layer of skin destroys normal sweat and oil glands. Second, the outer layer of the skin usually has fatty substances that moisten the skin. In scleroderma, this outer layer often gets thinner so there is less oil. Finally, in the early phase of scleroderma inflammation in the skin release histamine and other chemicals that cause the skin to itch.
Without treatment, itching from inflammation normally occurs in the first 6 months to 2 years of the disease and then gets better as the inflammation disappears. However, when the skin is thickened and damaged then it may have the problem with itching for many years.
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These sores are called digital ulcers and are caused when excess collagen collects on the inside of blood vessels causing them to narrow and restrict blood flow. They occur most often on the fingertips but can develop anywhere on the finger or toe when the skin in stretched too tight. Sores can also appear at pressure points, like elbows. Sores may be very painful and can make it difficult to use your hands or other parts of your body.
This is a result of Raynaud’s Phenomenon. Raynaud’s is a change in your fingers caused by the excess collagen of Scleroderma that narrows blood vessels and reduces the flow of blood to body tissues and organs. Raynaud’s phenomenon is often times the earliest symptom of Scleroderma.
The color change is an exaggeration of what normally happens when hands are exposed to the cold. However, the normal cold response is a blotchy red and white pattern while the color changes of a Raynaud’s attack--- white, then blue or purple are very different. At the end of the attack, the fingers turn red which is the result of blood flowing back to the fingers in a rush. This may be painful.
Yes, many people have Raynaud’s attacks in their toes.
The most common medications are called vasodilators. Calcium channel blockers are the most effective. They stop the spasm of arteries in Raynaud’s phenomenon. A topical nitroglycerin cram can also be used.
For more severe Raynaud’s Phenomenon with digital ulcers intravenous prostaglandins are given.
Biofeedback has been shown to be helpful. Biofeedback uses a machine to give you information on the temperature of your hands. A biofeedback therapist can teach you skills for increasing the blood flow and warming the temperature of your hands.