Progressive Scleroderma: Types risk factors, causes and preventions.
Definition
Scleroderma is an autoimmune condition of the connective tissue
characterized by skin thickening, spontaneous scarring, blood vessel
disease, and varying degrees of inflammation, associated with an
overactive immune system. Scleroderma is characterized by the formation
of scar tissue (fibrosis) in the skin. This leads to thickness and
firmness of involved skin. This may also occur in internal organs such
as the kidney.
Types
There are two major classifications of scleroderma: localized
scleroderma and systemic sclerosis (SSc). Other forms or sub
classifications, each with its own characteristics and prognosis.
- Localized Scleroderma: the changes, which
occur in localized scleroderma, are usually found in only a few places
on the skin or muscles, and rarely spread elsewhere. Generally,
localized scleroderma is relatively mild. The internal organs are
usually not affected, and persons with localized scleroderma rarely
develop systemic scleroderma.
- Morphea is a form of localized scleroderma characterized by
waxy patches on the skin of varying sizes, shapes and color. The skin
under the patches may thicken. The patches may enlarge or shrink, and
often may disappear spontaneously. Morphea usually appears between the
ages of 20 and 50, but is often seen in young children.
- Linear scleroderma is a form of localized scleroderma which
frequently starts as a streak or line of hardened, waxy skin on an arm
or leg or on the forehead. Sometimes it forms a long crease on the head
or neck, referred to as en coup de sabre because it resembles a saber or
sword wound. Linear scleroderma tends to involve deeper layers of the
skin as well as the surface layers, and sometimes affects the motion of
the joints, which lie underneath. Linear scleroderma usually develops in
childhood. In children, the growth of involved limbs may be affected.
- Systemic scleroderma (systemic sclerosis): The
changes occurring in systemic scleroderma may affect the connective
tissue in many parts of the body. Systemic scleroderma can involve the
skin, esophagus, gastrointestinal tract (stomach and bowels), lungs,
kidneys, heart and other internal organs. It can also affect blood
vessels, muscles and joints. The tissues of involved organs become hard
and fibrous, causing them to function less efficiently.
- In diffuse scleroderma, skin thickening occurs more rapidly and
involves more skin areas than in limited disease. In addition, people
with diffuse scleroderma have a higher risk of developing “sclerosis” or
fibrous hardening of the internal organs.
- In limited scleroderma, skin thickening is less widespread,
typically confined to the fingers, hands and face, and develops slowly
over years.
Risk factors
- Because of the known association of certain genes with scleroderma,
it may be that combinations of exposures to environmental factors and
possibly certain viruses may trigger the development of scleroderma in
genetically susceptible people.
- The precise interplay of pathology leading to blood vessel damage, scar and collagen accumulation, and autoimmunity is unclear.
Causes
- Scleroderma results from an overproduction and accumulation of
collagen in body tissues. Collagen is a fibrous type of protein that
makes up your body’s connective tissues, including your skin.
- Doctors aren’t certain what prompts this abnormal collagen
production, but the body’s immune system appears to play a role. In some
genetically susceptible people, symptoms may be triggered by exposure
to certain types of pesticides, epoxy resins or solvents.
Symptoms
Scleroderma’s signs and symptoms vary, depending on which parts of your body are involved:
- Skin. Nearly everyone who has scleroderma
experiences a hardening and tightening of patches of skin. These patches
may be shaped like ovals or straight lines, or cover wide areas of the
trunk and limbs. The number, location and size of the patches vary by
type of scleroderma. Skin can appear shiny because it’s so tight, and
movement of the affected area may be restricted.
- Fingers or toes. One of the earliest signs of
scleroderma is an exaggerated response to cold temperatures or emotional
distress, which can cause numbness, pain or color changes in the
fingers or toes. Called Raynaud’s disease, this condition also occurs in
people who don’t have scleroderma.
- Digestive system. In addition to acid reflux, which
can damage the section of esophagus nearest the stomach, some people
with scleroderma may also have problems absorbing nutrients if their
intestinal muscles aren’t moving food properly through the intestines.
- Heart, lungs or kidneys. Scleroderma can affect the
function of the heart, lungs or kidneys to varying degrees. These
problems, if left untreated, can become life-threatening.
Diagnosis and Tests
Because scleroderma can take so many forms and affect so many different areas of the body, it can be difficult to diagnose.
- He or she may remove a small tissue sample (biopsy) of the affected
skin so that it can be examined in the laboratory for abnormalities
- Doctors may also suggest breathing tests (pulmonary function tests), a CT scan of lungs and an echocardiogram of heart
Treatment and Medication
No drug has been developed that can stop the underlying process of
scleroderma- the overproduction of collagen. But a variety of
medications can help control scleroderma symptoms or help prevent
complications. To accomplish this, these drugs may:
- Dilate blood vessels.Blood pressure medications
that dilate blood vessels may help prevent lung and kidney problems and
may help treat Raynaud’s disease.
- Suppress the immune system.Drugs that suppress the immune system, such as those taken after organ transplants, may help reduce scleroderma symptoms.
- Reduce stomach acid.Medications such as omeprazole (Prilosec) can relieve symptoms of acid reflux.
- Prevent infections.Antibiotic ointment, cleaning
and protection from the cold may help prevent infection of fingertip
ulcers caused by Raynaud’s disease. Regular influenza and pneumonia
vaccinations can help protect lungs that have been damaged by
scleroderma.
- Relieve pain.If over-the-counter pain relievers don’t help enough, you can ask your doctor to prescribe stronger medications.
Surgery
Used as a last resort, surgical options for scleroderma complications may include:
- If finger ulcers caused by severe Raynaud’s disease have developed gangrene, amputation may be necessary.
- Lung transplants.People who have developed high
blood pressure in the arteries to their lungs (pulmonary hypertension)
may be candidates for lung transplants.
Therapy
Physical or occupational therapists can help you to:
- Manage pain
- Improve your strength and mobility
- Maintain independence with daily tasks
Prevention
There is no prevention method or diet to avoid or decrease the risk of scleroderma.
