It is interesting that the word scleroderma means literally "hardening of the skin" which points out to the one of the possible physical effects of the disease.
Localized scleroderma is referred to as morphea or linear scleroderma and does not affect the internal organs. The cause of the condition is unknown but there are several treatment options!
Incidence of the condition
Several researches have came to the data that the incidence of morphea has been estimated as approximately 25 cases per million population per year. Of course- it is important pointing out that the actual incidence is likely higher because many cases may not come to medical attention. Two thirds of adults with morphea present with plaque-type lesions, with generalized, linear, and deep variants each accounting for approximately 10% of cases. Linear morphea predominates in pediatric patients, followed by the plaque (25%) and generalized (5%) subtypes.
Mechanism of the condition
The most important thing to know about the mechanism of this disease is that overproduction of collagen by fibroblasts in affected tissues is common to all forms of morphea. The exact mechanism by which these fibroblasts are activated is unknown. Proposed factors involved in the pathogenesis of morphea include:
- endothelial cell injury
- immunologic activation
- inflammatory activation
- dysregulation of collagen production
An autoimmune etiology is supported by the frequent presence of autoantibodies in patient with this disease!
Symptoms and types of the condition
There are two forms of scleroderma:
Every patient should know that localized scleroderma affects mainly the skin and- it happens in different areas of the body. It may also affect muscles and bone, but it does not affect internal organs- which is the most important thing to know about this form of sclerosis. Good thing about this form is the fact that it is usually not as severe as generalized scleroderma.
There are two types of localized scleroderma:
There are two types of localized scleroderma:
Early morphea scleroderma has an inflammatory stage, followed by one or more slowly enlarging patches or plaques which are most commonly oval in shape and vary in size. What's also characteristic- they have a yellow center and are surrounded by a violet colored area. If we talk about the level of these skin changes- we should know that they may be depressed or slightly elevated and are seen more often on the trunk, but may also occur on the face and extremities.
Limited or localized scleroderma is commonly referred to by the acronym CREST, whose letters are the first initials of characteristics that are typically found in this syndrome:
Every patient should know that in this condition, mineral crystal deposits form under the skin and this happens usually around the joints. Problem is also that some small skin ulcers filled with a thick white substance may form over the deposits which could be extremely difficult to treat.
- Raynaud’s phenomenon
This is famous syndrome in which the fingers of both hands are very sensitive to cold, and they remain cold and blue colored after exposure to low temperatures. Although for many years experts couldn’t explain this phenomenon but now- experts agree that it is caused by abnormal changes in the small arteries and capillaries, which result in constriction and a temporary interruption of circulation, usually in the fingers.
- Esophageal motility dysfunction
This serious condition happens when the muscles in the esophagus become scarred by scleroderma and do not contract normally. Patient should know that this can cause severe heartburn and other symptoms of gastroesophageal reflux disorder.
Although not too many people have heard about this syndrome- the fact is that it is nothing more then simple stiffness and tightening of the skin of the fingers, a classic symptom of scleroderma.
In this situation, dilation of small vessels and capillaries cause numerous flat red marks on the hands, face, and tongue.
Generalized - systemic sclerosis
Important thing to know is that generalized morphea may involve almost the entire skin surface, which could sometimes turn into a really serious condition. An uncommon form of morphea is the guttate form which is characterized by multiple, small, chalk-white spots which vary in size from 1 to 10mm in diameter. The violet-colored line may surround all the spots and in cases of long duration the line may be brown or grayish. Although the fact is that localized morphea may last from a few months to many years- good thing is that a large proportion of morphea patients improve spontaneously.
Possible causes of the condition
Although some experts are claiming different- the fact is that the cause of morphea is still unknown. Experts have not uncovered any consistent etiologic factors which could possibly lead to all those different morphea subtypes which often coexist in the same patient. Some of the possible causes could be:
Several researches done in the past have shown that Morphea can occur at the localization of previous radiation therapy for different malignancies but most commonly- for breast cancer. Skin changes are developing from 1 month up to more than 20 years after irradiation.
Not too many people know that infections, such as Epstein-Barr virus infection, varicella, measles, and borreliosis, have been reported to trigger the onset of morphea and that’s why- they have been proposed as possible triggers. It is also important to point out that the most extensive literature focuses on Borrelia burgdorferi as a possible etiologic agent for morphea. However- the problem is that several more recent studies found no serologic evidence of Borrelia infection in patients with morphea. Morphea-like lesions have also been reported to occur after tuberculosis TBC and tetanus vaccinations.
Some morphea patients report a history of local trauma directly preceding the onset of disease. Excessive physical exertion triggers eosinophilic fasciitis in approximately half the cases.
A few familial cases of morphea have been reported, most commonly the disabling pan-sclerotic subtype.
Diagnosis of the condition
- Immunoglobulin G and immunoglobulin M
It is well known that polyclonal increases in both antibody types may occur, especially in patients with linear and deep morphea.
Serum autoantibodies are commonly present in all types of morphea which could be extremely useful in making the right diagnosis!
Every patient should know that radiography may be helpful in cases of linear or deep morphea in which involvement of the underlying bone is suspected.
- Histological Findings
The histological findings of morphea and systemic sclerosis are similar, with a fundamental process of thickening and homogenization of collagen bundles.
Therapy of the Morphea Scleroderma
Although there is not yet a cure for scleroderma, there are many medications that help control it and patients should get familiar with all the options! Only problem is that some work well for some people and not at all for others.
- Aspirin and steroids
Several researches done in the past have proven that Aspirin may be used in large doses to treat joint pain and swelling. It may be used to treat joint pain and swelling. Steroids could also be helpful because they are in fact nothing more then man-made forms of naturally occurring hormone in the body.
- Antacids for stomach problems
The fact is that medications called antacids may be used to treat heartburn and to protect the esophagus.
- Blood pressure medication
Blood pressure medication may be used to treat high blood pressure which is sometimes the co-occurring problem of scleroderma. Every patient should know that high blood pressure is known as the silent disease because it has no symptoms. Drugs that increase blood flow to fingers and toes may be used to treat Raynaud's phenomenon and that’s the most important effect!
There is no doubt- it is proven that regular exercise helps improve overall health and fitness. That’s why -for people, who have scleroderma, it also helps keep the skin and joints flexible, maintain better blood flow, and prevent contractures. It is also important to keep the joints healthy because swollen and painful joints from stresses and strains that can make them hurt more. Lifting or carrying heavy objects, for example, can strain and hurt your joints and that’s why- it should be strongly avoided!
- Skin protection
Well, everyone knows that the goal of skin protection is to keep a good supply of blood flowing to skin, and of course- to protect skin from injury. Keeping body warm helps open the blood vessels in arms, hands, legs, and feet and prevent any injury from cold to occur!