Scleroderma: Causes, Symptoms, Diagnosis and Treatment
by MedPlus Team, August 12, 2019
Scleroderma also known as systemic sclerosis, it is not a contagious, malignant or cancerous disease but, it is a chronic connective tissue disease normally categorized as an autoimmune rheumatic disease.
In simple forms Sclero=hard and Derma=skin. Therefore, this means hardening of the skin is one of the visible symptoms of the disease.
In scleroderma, the bodies own immune system attacks the body tissues end up by overproduction of collagen, it is a protein that forms the basis of connective tissue. Hence, results in thickening, fibrosis, and scarring of tissue, though it is not a contagious disease. This may run in families, however, it frequently occurs in people without any family history of the disease. This ranges from mild to potentially fatal. Out of 3, 1 person with the condition may develop severe symptoms. However, the below-mentioned symptoms may vary from individual to individual, this may affect any part of the body or the whole body system.
Causes and Symptoms
Causes of scleroderma are not known, to find out, the research is still going on.
The symptoms of scleroderma in the initial stage includes:
1. Changes in the fingers and hands.
4. Puffiness due to sensitivity to cold or emotional stress
5. Swelling in the hands and feet, especially early morning
Overal symptoms of this condition may include:
1. Connective tissue is deposited with calcium
2. Narrowing of the blood vessels to the hands and feet called Raynaud’s disease
3. Discomfort to the esophagus, that links the throat and stomach
4. Thick and tightness in the fingers
5. Redness on the face and hands
Types of Scleroderma
Scleroderma varies from mild to severe. The main two types are localized and systemic scleroderma:
1. Localized Scleroderma:
This is the mild form of scleroderma, this does not affect the internal organs, this again categorized into:
Morphea and Linear scleroderma
a. Morphea: This includes oval-shaped patches of lighter or darker skin, which may be itchy, hairless and shiny. The shapes have a purple border and they remain white in the middle.
b. Linear Scleroderma: This includes bands or streaks of hardened skin on the limbs. This rarely affects the head and face. This can also affect bones and muscles.
2. Systemic Scleroderma: This affects the circulation of the blood and the internal organs.
The two main types of systemic Scleroderma includes:
a. Limited Cutaneous Systemic Sclerosis Syndrome or CREST: This is the least severe type of systemic scleroderma, this affects the skin of the hands, legs, feet, face, and lower legs and arms, also this may affect the vessels, lungs, and the digestive tract.
This condition, at times also known as CREST syndrome because the symptoms of the CREST form the acronyms:
C: Calcinosis-calcium deposits in tissues and under the skin
R: Raynaud’s disease-Circulatory problems due to narrowed blood vessels in the hands and feet
E: Esophageal problems-includes GERD
S: Sclerodactyly-thick skin on the fingers
T: Telangiectasias-enlarges blood vessels exhibiting as red spots
b. Diffuse System Sclerosis: In this, thickening of the skin usually affects the area from the hands to above wrists and also affects the internal organs, not only this, but the patients with this condition may also experience weight loss, weakness, fatigue, difficulty to breath, and swallow.
The diagnosis for Scleroderma is a bit difficult due to gradual and different forms of symptoms on the different parts of the body.
The physician would carry out a physical examination and some tests, the patient may also be referred to a rheumatologist- a specialist in connective and joint tissue diseases.
The following are the required tests to be carryout:
1. Diagnosing the skin under a microscope for changes in the small blood vessels, capillaries, around the fingernails
2. Skin biopsy-Removes cells or skin samples from the body
3. Blood tests to evaluate the levels of certain antibodies
4. The doctor would evaluate for the signs of skin thickening, GERD, shortness of breath, joint pain, and calcium deposits. They may also check for heart, lung, or gastrointestinal complications
There is no cure for scleroderma and no medication can stop the over-release of collagen. However, organ system complications can be treated to minimize damage and maintains functionality.
Localized Scleroderma may heal on its own, some medications can help control the symptoms and helps to prevent the complications.
Finally, the target is to relieve the symptoms, to prevent the condition from worsening and also to detect and treat complications as soon as possible and also to minimize disabilities.
Treatment purely depends on the symptoms of the affected individual.
1. Immune suppressants may calm or suppress the immune system
2. Blood pressure medication may help to dilate the blood vessels, this helps to decrease the problems with the internal organs such as lungs, kidneys and also helps to treat Raynaud’s disease
3. Physical therapy helps to manage pain, improves the mobility and boosts strength to the body
4. Ultraviolet Light Therapy-This may help to better the condition and the appearance of the skin
Lets hope, the scientists may find treatment and permanent cure for this condition!