This action might not be possible to undo. Are you sure you want to continue?
What is Scleroderma?
Derived from Greek words skleros, meaning hard, and derma, meaning skin. Literally means hard skin. A chronic autoimmune connective tissue disease that may have multi-organ involvement when systemic and has an unpredictable course that affects the circulation and blood vessels; causing inflammation and autoimmunity; and results in fibrosis (scarring) on the skin, lungs, and other organs. Characterized by alterations of the microvasculature, disturbances of the immune system and by massive deposition of collagen and other matrix substances in the connective tissue. Scleroderma is often referred to as a single disease, but really a symptom of a group of diseases involving the abnormal growth of connective tissue, which supports the skin and internal organs.
and cartilage. . Connective tissue disease is one that affects tissues such as skin. joints. or fibrous tissue.What is Scleroderma? Scleroderma is both a rheumatic disease and a connective tissue disease: Rheumatic disease refers to a group of conditions characterized by inflammation or pain in the muscles. tendons.
. can affect the whole body.The Different Types of Scleroderma The group of diseases known as scleroderma fall into 2 main classes: localized and systemic Localized Scleroderma affect only certain parts of the body. or Systemic Sclerosis. Systemic Scleroderma. Both groups include subgroups which may be broken down or referred to in different ways.
Common Classifications of Scleroderma .
Localized Scleroderma Localized Scleroderma is limited to the skin and related tissues and. . Localized conditions often go away on their own over time. the localized form never progresses to the systemic form of the disease. the muscle below. in some cases. Internal organs are not affected. and usually. but damage can be permanent.
Linear Scleroderma-refers to a form of the condition characterized by a single line or band of thickened or abnormally colored skin. or generalized.Localized Scleroderma Generally. there are two recognized types of Localized Scleroderma: Morphea-refers to local patches of scleroderma which can be either localized. where the skin patches spread over larger areas of the body. limiting itself to one or several patches. .
Localized Scleroderma Morphea First signs of this form of the disease are reddish patches of skin that thicken into firm. and they sweat very little and have little hair growth. The center of each patch becomes ivory colored with violet borders. Patches appear most often on the chest. stomach. and sometimes. oval-shaped areas. arms. . appear on the face. and back. and legs.
. the line runs down an arm or leg. and may also involve muscle or bone. The French term en coup de sabre. Usually. but in some people it runs down the forehead.Localized Scleroderma Linear Scleroderma Signs of this condition are characterized by a single line or bands of thickened or abnormally colored skin. or ³sword stroke´ is sometimes used to describe this highly visible line.
but also involves the tissues beneath. it is also broken down into a third subset called Systemic Sclerosis Sine in which patients do not have any overt skin thickening. the blood vessels. this form of the disease is broken down into two major types: Limited Cutaneous Scleroderma and Diffuse Cutaneous Scleroderma. Sometimes. Generally. but show other manifestations of the disease. and the major organs.Systemic Scleroderma Systemic Sclerosis is the form of the disease that not only includes the skin. .
lower arms. as well as severe lung problems. and Telangiectasia. Esophageal dysfunction. Because of the predominance of CREST symptoms in people with limited systemic scleroderma. CREST syndrome is an acronym for a collection of symptoms that occur to some degree in all people with systemic sclerosis. Raynaud¶s phenomenon. many use the term CREST syndrome to refer to this form of the disease. Sclerodactyly. and legs. followed by Telangiectasia and Calcinosis. face. . even if the skin thickening remains limited.Systemic Scleroderma Limited Cutaneous Scleroderma Typically comes on gradually and affects the skin only in certain areas: the fingers. Gastrointestinal involvement also commonly occurs. People with this form often have all or some of the symptoms that some doctors call CREST. Most people experience Raynaud¶s phenomenon for years before skin thickening starts. Symptoms include: Calcinosis. hands.
Systemic Scleroderma Limited Scleroderma/CREST Syndrome .
and trunk and on the skin above elbows and knees. which can be detected by x-ray. hands. Deposits are typically found on the fingers. When the deposits break through the skin. . painful ulcers can result.Calcinosis Calcinosis: Formation of calcium deposits in the connective tissues. face.
the hands or feet turn white and cold. they become red. or gangrene. leading to ulcers. . scars. then blue. As the vessels contract. Fingertip tissues may suffer damage. As blood flow returns.Raynaud¶s Phenomenon Raynaud¶s phenomenon: Condition in which the small blood vessels of the hands or feet contract in response to cold or anxiety.
the result can be chronic heartburn and inflammation. In the lower esophagus. .Esophageal Dysfunction Esophageal dysfunction: Impaired function of the esophagus that occurs when smooth muscles in the esophagus lose normal movement. or other symptoms of gastroesophageal reflux disorder (GERD) . the result can be difficulties in swallowing. In the upper and lower esophagus.
. The skin may also appear shiny and darkened. The abnormal build-up of fibrous tissue in the skin can cause the skin to tighten so severely that the fingers curl and lose their motility. resulting from deposits of excess collagen within skin layers.Sclerodactyly Sclerodactyly: Thickened and tight skin on the fingers. with hair loss. The condition makes it harder to bend or even straighten the fingers.
face. . and tongue. Although not painful. in which small red spots appear on the hands. these red spots can create cosmetic problems.Telangiectasia Telangiectasia: A condition caused by the swelling of tiny blood vessels.
affecting the hands. and stomach in a symmetrical fashion. Diffuse form may also cause damage to internal organs such as the intestines. this is referred to as mixed connective disease. and during this phase. This can become severe and even life-threatening. people often enter a stable phase lasting for varying lengths of time. upper arms. Diffuse scleroderma can overlap with other autoimmune diseases. heart. chest. including systemic lupus erythematosus and polymyositis. face. After the first 3-5 years. In such cases.Systemic Scleroderma Diffuse Cutaneous Scleroderma Typically comes on suddenly and begins with skin thickening on hands that spreads quickly and over much of the body. many of the symptoms will subside. lungs. . and kidneys. upper legs.
. Microchimerism has also been proposed as a cause of scleroderma in which fetal cells are left in the mother¶s body after giving birth.What Causes Scleroderma? The exact cause of scleroderma is still unknown. This theory arose from the fact that the disease occurs mostly in women of childbearing age. Factors can lead to immunological disturbances and vascular changes. and symptoms resemble those of graftversus-host disease (GVHD) which occurs in bone marrow transplant patients who have received cells from another person. but evidence shows that the disease may be caused by a number of genetic abnormalities which are triggered by environmental factors.
and HLA-DQB2. including genetic. Fibrosis can be caused by profibrotic cytokines. are involved in systemic sclerosis. One theory states that antigens from the human leukocyte antigen (HLA) histocompatability complex. autoimmunologic. and connective-tissue growth factor. environmental. Some data suggest that apoptosis and the generation of free radicals may also be involved in the pathogenesis of systemic sclerosis. activated infiltrate early. HLA-DR3. including HLA-B8. The activation of the immune system is of great importance in the pathogenesis of systemic sclerosis. a B-cell transduction molecule. infiltrate the skin. including transforming growth factor-beta (TGF-beta). which results in decreased fibrosis in animal models. Antigen-activated T cells. vascular. . as deficiency of CD19. B cells may also contribute to fibrosis. The vasculopathy may be linked to TGF-beta and PDGF. interleukin-4 (IL-4). HLA-DR52. HLA-DR5. while the diminishing of lesional cutaneous blood vessels can be attributed to antiendothelial cell autoantibodies. and microchimeric factors are involved in systemic sclerosis pathogenesis.Pathophysiology As stated before. platelet-derived growth factor (PDGF). and produce the profibrotic cytokine IL-4. different factors.
a substantial female predominance exists. dSSc occurs equally in males and females. Pulmonary hypertension leads to 12% of systemic sclerosis±related deaths. with a female-to-male ratio of 10:1. Generally. . Age Systemic sclerosis usually appears in women aged 30-40 years.08 persons are affected per 1 million. In approximately 85% of cases.Epidemiology Frequency United States-Systemic sclerosis is a rare disease. However.000 people each year. Race No apparent racial predominance exists. Systemic sclerosis is rare in the resident population of Japan and China. renal and lung changes are responsible for death in patients with systemic sclerosis. Sex Overall. Diffuse systemic sclerosis (dSSc) occurs more often in black women than in caucasian women. systemic sclerosis is rare in the resident population of Japan and China.3-10 people per 1 million. systemic sclerosis develops in individuals aged 20-60 years. Lung fibrosis and heart changes are responsible for 9% of systemic sclerosis±related deaths. However. Cases also are observed in children and in the elderly population. and it occurs in slightly older men. The limited form of systemic sclerosis (lSSc) has a strong female predominance. Another analysis showed that men tend to have diffuse disease and women to have calcinosis. Mortality/Morbidity The mortality rate is increasing in the United States and Europe. Systemic sclerosis is diagnosed in approximately 67 male patients and 265 female patients per 100. with a female-to-male ratio of 3-6:1. International-Systemic sclerosis is estimated to occur in 2. as many as 3.
This action might not be possible to undo. Are you sure you want to continue?
We've moved you to where you read on your other device.
Get the full title to continue listening from where you left off, or restart the preview.