What is Scleroderma?
Scleroderma is a group of progressive and rare diseases that comprise the tightening and hardening of the skin as well as connective tissues – the fibers that provide the support and framework for the body.
Localized scleroderma upsets only the skin. Systemic scleroderma can also harm internal organs such as the lungs, heart, digestive tract as well as kidneys.
Scleroderma may happen to anyone at any time in any geographical area. But, the disease affects women more than men and is most common between the ages of 30 and 50. Scleroderma may run in families, but in the majority of cases occurs without any known family propensity for the disease. Scleroderma is not believed to be contagious, but it may affect greatly an individual’s self-esteem as well as the ability to accomplish everyday tasks.
Scleroderma Symptoms
The symptoms of scleroderma vary, and are dependent on the organ system involved. The diagnosis can be problematic since some of the initial symptoms are common in the general populace and are not always linked with scleroderma. The more prevailing symptoms and signs of scleroderma consist of:
Raynaud’s phenomenon
This is an inflated reaction to temperatures that are cold or emotional anguish, with this condition constricting the small blood vessels in the feet and hands and causes pain, as well as color changes in the toes and the fingers.
Gastroesophageal reflux disease or GERD
Additionally to acid reflux that can damage the segment of the esophagus nearest the stomach, the individual can also have difficulties with nutrients absorption if the muscles of the intestines are not moving food thru the intestines appropriately.
Skin changes
These deviations can include hands and fingers that are swollen; thickened areas of skin, predominantly on the fingers; and skin that is tight around the face, mouth or hands. The skin may appear shiny due to it being so tight and moving of the areas that are affected can be constrained.
Scleroderma Localized
The main 2 diversities of localized scleroderma, that affects simply the skin, are notable by very distinctive symptoms and signs:
Morphea
This type of scleroderma features oval-shaped patches of thickened skin that are white in the middle, with a border that is purple. Patches of skin can appear in only a few areas on the body or be more widespread, appearing all over the body. Morphea normally fades out after 3 to 5 years, but can maintain areas of darkened skin where the thickened skin areas appeared.
Linear scleroderma
This type of scleroderma is most common in children and features streaks or bands of hardened skin on one or both legs or arms, or on the forehead. This type of scleroderma normally appears mainly on one side of the body.
With localized scleroderma, the primary signs of disease can be the presence of Raynaud’s phenomenon which may develop several years prior to noticing any other symptoms.
Scleroderma Systemic
This type of scleroderma is also referred to as systemic sclerosis, and affects not only the skin but also blood vessels as well as internal organs. Subcategories are demarcated by the portions of the body that is affected. One variation is known as limited scleroderma or CREST syndrome.
With this type of scleroderma, changes in the skin can occur suddenly as well as progressively get worse during the first 1 to 2 years of the disease. After that time, changes normally level off or subside all together and often even resolve without treatment.
Scleroderma Causes
Scleroderma can be the result of over-production as well as buildup of collagen in body tissues. Collagen is a type of fibrous protein which makes the body’s connective tissues, including the skin.
Medical professionals are not certain what are triggering this abnormal production of collagen but they believe that the immune system seems to play a role. For some unknown reasons, the immunity system turns against the body, causing inflammation as well as the over-production of collagen.
There are several factors which seem to escalate the jeopardy of certain kinds of scleroderma:
Ethnicity and race – definite groups of individuals are much more possible to develop scleroderma than others:
African-Americans
Scleroderma that is systemic is most likely in African-Americans than Americans of European ancestry. As well, African-Americans who have scleroderma that is systemic are most possible to develop austere lung problems.
Native Americans
Native Americans of the Choctaw Nation living in the area of Oklahoma are 20X as probable as the general populace to develop scleroderma that is systemic. What is especially odd is that this increased possibility does not apply to those Choctaws who live in Mississippi.
Gender – Scleroderma happens at the very least 4X as often with females as with males.
Environmental causes – contact with various substances can be associated with the expansion of scleroderma. Instances include:
- Certain chemotherapy medications
- Silica dust that is common in rock quarries as well as coal mines
- Certain industrial solvents – for instance paint thinners
Scleroderma Complications
Scleroderma has complications that are a collection from very mild to very severe. Some can turn out to be life-threatening. These complications include:
Circulatory
The diversity of Raynaud’s phenomenon that happens with scleroderma may be so serious that the constrained flow of blood can do permanent damage to the tissues on the fingertips, triggering skin sores or ulcers and pits. In several cases, gangrene as well as amputation can follow.
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Lung
The scarring of tissues in the lung – known as pulmonary fibrosis –causes reduced functioning of the lungs, reducing the capacity to breathe as well as the reduction to tolerate exercise. The individual might even develop blood pressure that is high in the arteries of the lungs and this is referred to as pulmonary hypertension.
Heart
Scarring of the heart tissue can increase the risk of heartbeats that are abnormal (arrhythmias) as well as causes congestive heart failure. It also can cause inflammation of the membranous sac surrounding the heart – known as pericarditis.
Kidney
When scleroderma disturbs the kidneys, the individuals develop a blood pressure which is elevated as well as an increased level of urine protein. More grave effects of complications to the kidneys can include renal crisis involving the sudden increase in blood pressure as well as rapidly failing kidneys.
Digestive
Scleroderma may affect the upper as well as lower digestive tracts. Digestive troubles connected with scleroderma may lead to acid reflux disease as well as in difficulty swallowing – many describe the feeling as if the food gets stuck halfway down the esophagus. There are also bouts of constipation interchanging with episodes of diarrhea.
Dental
The severe constriction of the facial skin may cause the mouth to become smaller as well as narrower, which can make it very hard to brush the teeth or to even have them cleaned professionally. Individuals who have scleroderma can often limit the production of normal amounts of saliva, so the decay risk grows even more. Additionally, acid reflux can destroy enamel of the teeth and this changes gum tissues that can cause the teeth to become loose or to even fall out.
Scleroderma Treatment
The primary care physician will normally conduct the following tests:
Blood tests
Individuals with scleroderma normally have blood levels of certain antibodies that are elevated and produced by the immune system.
Assessment of the skin
The physician evaluates the condition of the skin in 17 exact areas to define the level of involvement of the skin, and provide clues to the possibility that the individual might also have changes happening to organs internally that can be life-threatening.
Tissue samples
The physician can remove samples of tissues – known as biopsy – of the skin that is affected to be examined in the lab for any abnormalities.
Based on the outcome of these initial evaluations, the physician can as well recommend other diagnostic type tests to identify any heart, kidney, gastrointestinal or lung complications linked to scleroderma.
Scleroderma does not have a cure at this time – no medication will stop this over-production of the collagen. But the generalized types of scleroderma from time to time can resolve on their own. And there are assortments of drugs that can aid in the control of the symptoms and signs of scleroderma or help to avert any complications.
Scleroderma Medications
Blood vessel dilating
Blood pressure drugs that dilate blood vessels can aid in preventing kidney and lung problems and help to treat Raynaud’s disease.
Immune system suppressing
Medications that subdue the immune system such as ones used after organ transplants can help to decrease scleroderma symptoms.
Scleroderma Therapy
Occupational and physical therapy
There are therapists who may help individuals to manage the pain, improve mobility and strength, and work on accomplishing daily tasks in order to maintain freedom.
Cosmetic measures
The presence of skin lesions linked with scleroderma can be aided by contact with ultraviolet light. Laser surgery also can help to eliminate or camouflage these lesions.
Scleroderma Surgery
Amputation
When ulcers of the finger due to severe Raynaud’s disease develop gangrene, amputation will become required.
Lung transplants
Individuals who have developed blood pressure which is high in arteries of the lungs or pulmonary hypertension can be subjects for transplants of the lungs.
Scleroderma Prognosis
As with every chronic disease, learning to live with scleroderma can place an individual on a roller coaster of emotions. The individual must remember that the physical health has a direct impact on the mental and emotional health. Denial, anger as well as frustration are common with the prognosis of chronic illnesses.
Localized scleroderma almost all the time carries a very good prognosis as well as a life span that is normal. Even scleroderma that is localized can cause some serious problems in children, which include growth that is impaired, limb imbalance as well as problems in bending and flexing muscles.
The prognosis for individuals with scleroderma systemic has improved greatly thru the years. 10-year survival rates rose from 54 percent in 1972 to 66 percent in 2001.
The usual cause of death connected to systemic scleroderma has also altered. The share of deaths from crises with the kidney has significantly fallen, while the share of deaths from pulmonary fibrosis has enlarged. Currently, complications of the lungs account for 60 percent of scleroderma-connected deaths.
Scleroderma Pictures
