What Is Scleroderma?
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Scleroderma - Symptoms, Causes, Types, Diagnosis, and Treatment

Published on Oct 18, 2022   -  5 min read


Scleroderma is a rarely occurring, chronic, autoimmune connective tissue disease that results in hardening and tightening of the skin. Read on to know more.

What Is Scleroderma?

A long-lasting rheumatic disease that affects the skin, connective tissues, and internal organs are scleroderma, which is often characterized as limited or diffuse, depending on the degree of skin involvement. Autoimmune diseases tend to be caused due to the immune system attacking the body cells and tissues, which weakens and destroys organs in the body.

What Are the Causes of Scleroderma?

The immune system causes the body to produce large amounts of the protein collagen, which is an important part of the skin. This causes the skin to get thick and tight, and scars form on the lungs and kidneys.Most likely, scleroderma is caused by a combination of factors, including immune system problems, genetics, and environmental triggers. Research suggests that scleroderma symptoms may be triggered due to exposure to certain viruses, medications, and drugs.

What Are the Types of Scleroderma?

1. Localized Scleroderma - This type of scleroderma affects only the skin and not the major organs. It consists of three types:

  • Circumscribed Morphea: Circumscribed morphea is characterized by either a single or a few discolored patches. These patches appear pale yellow in color with a red border and are of varying sizes. Active or symptomatic lesions are typically enlarged and become flat when treated and asymptomatic.
  • Generalized Morphea: Generalized morphea is characterized by several discolored patches across different anatomical areas. These patches are not distinct from each other. They may blend into each other and appear as tight and thick lesions.

  • Linear Scleroderma: Linear scleroderma is characterized by the appearance of a single, tight, thick band in a single body area that may be interspersed with morphea patches. It may even affect the muscles and bones and cause disability of the limbs when it appears in the extremities.

2. Systemic Scleroderma - This type of scleroderma affects the skin and may affect the tissues underlying it, including the blood vessels and major organs, like the gastrointestinal tract, kidneys, heart, and lungs. It consists of the following three types:

  • Limited Cutaneous Scleroderma: The commonest type, limited scleroderma causes the tightening and hardening of the fingers and occasionally the forearms, hands, or face. These patients are able to lead normal lives, although some may develop concurrent issues like heartburn, musculoskeletal pain, and Raynaud’s phenomenon.

  • Diffuse Cutaneous Scleroderma: Diffuse scleroderma affects large areas of the body and is characterized by the thickening of the skin (accompanied by an itching sensation and dryness) over these areas because of excess collagen production. Damage to organs like the heart, kidneys, gastrointestinal tract, and lungs may occur in this type of sclerosis. The condition can be pretty severe and life-threatening.

  • Sine Sclerosis: Sine sclerosis demonstrates systemic symptoms and illnesses seen in limited or diffuse scleroderma-like Raynaud’s phenomenon, but the skin symptoms are absent.

What Are the Signs and Symptoms of Scleroderma?

The following are the manifestations of scleroderma:

Who Is at an Increased Risk of Getting Scleroderma?

Although anyone can get scleroderma, some groups are at a higher risk of getting affected. They include:

  • Sex - Scleroderma is more common in women than men.

  • Age - It occurs most commonly between the ages of 30 and 50 and is more common in adults than children.

  • Race - Scleroderma can affect people of all races and ethnic groups, but African- Americans are more severely affected.

How Is Scleroderma Diagnosed?

In addition to the patient's symptoms, medical history, and thorough physical examination, various tests are conducted by the doctor to aid in the diagnosis of scleroderma. These include -

1. Antinuclear Antibody Testing: Elevated levels of immune factors, known as antinuclear antibodies found in 95% of scleroderma patients. It checks the presence of autoantibodies (blood proteins) that are present in the blood.

2. Pulmonary Function Test: Rules out the functioning of the lungs.

3. Electrocardiogram : The ECG test is carried out to check whether the disease has affected the heart. Detects the activities of the heart and checks the abnormal rhythm of the heart.

4. Echocardiogram: To evaluate complications like hypertension and congestive heart failure.

5. Gastrointestinal Tests:

  • Manometry can measure the strength of the esophageal muscles.

  • An endoscopy can be performed to view the esophagus.

6. Kidney Function Tests: These are done to evaluate the function of the kidneys and whether the disease has affected the kidneys, which can result in an increase in blood pressure as well as the leakage of protein into the urine. The serious form is called scleroderma renal crisis.

What Are the Complications of Scleroderma?

  • Digestive disorders.

  • Pulmonary hypertension.

  • Wound infections.

  • Renal failure.

  • Cardiovascular disorders.

  • Muscle pain and joint pain.

  • Inability to open mouth due to tightening of facial skin and narrowing of mouth.

  • Lungs disease.

  • Fingertips ulceration.

What Is the Treatment of Scleroderma?

Managing the symptoms is the aim of treatment, and a combination of approaches is often needed to treat and manage the disease effectively.

  • Pain relief by non-steroidal anti-inflammatory drugs or corticosteroids.

  • Maintaining muscle strength through physical exercise and therapies.

  • Controlling blood pressure and increasing blood flow with medications.

  • Improving emotional state through counseling, motivation, and other measures.

  • Skin Treatments: For localized scleroderma, topical medications are beneficial, and moisturizers prevent the drying of the skin. Nitrates help to relax the smooth muscles, causing arteries to dilate.

  • Digestive Medications: A variety of medications can be used to prevent digestive problems and heartburn, such as Antacids and Proton pump inhibitors.

  • Treatment of Lung Disease: In patients with scleroderma who have rapidly worsening pulmonary fibrosis, the drug Cyclophosphamide (Cytoxan), a form of chemotherapy, has been proven useful in recent research.

  • Surgery- If the complications cannot be resolved with minimally invasive therapies. Ulcers lead to gangrene, and amputation of the parts might be done.


Although there is no cure for scleroderma, the disease is often slowly progressive and manageable, and people can lead a healthy life. A multidisciplinary approach is most important for scleroderma patients because of the complications of the disease. Make sure to pursue regular activities, stay well-rested, seek the help of a therapist for coping and relaxation techniques, and reach out to support groups and trusted friends and family members. These simple acts can help to deal with the condition and stay positive.

Frequently Asked Questions


What Are the Initial Signs of Scleroderma?

The initial signs of scleroderma include -
- Hard, thickening, or tight skin.
- Hair loss and less sweating.
- Dry skin and itching.
- Changes in skin color.
- Stiffness of joints and difficulty moving them.
- Muscle weakness.
- Loss of tissue beneath the skin.
- Sores and pitted scars on the fingers.
- Visible blood vessels.


What Is the Mortality Rate in Scleroderma?

Scleroderma is a group of conditions causing the hardening and thickening of the skin. People with this condition have a normal life with only minor symptoms that are easy to manage. However, patients with this condition have a prognosis of between three and 15 years and do not have any fatal effects.


Is There a Cure for Scleroderma?

Presently, there is no cure for scleroderma. The treatment is primarily focused on managing the symptoms. As various symptoms appear in this condition, multiple systemic approaches are needed to manage the symptoms, ranging from symptoms related to the skin, joints, lungs, and muscles.


Is There a Cure for Scleroderma in Ayurveda?

There is no sure-shot treatment for scleroderma, and the treatment usually involves dealing with its symptoms. Ayurveda involves eliminating the root cause of the condition. Therefore, Ayurveda helps manage this condition by improving the body's immune system and nourishing the myeline-producing cells. Moreover, Ayurveda treatment focuses on identifying the root cause of illness called Ama, which means undigested food that produces toxins.


Who Is Most Likely to Get Scleroderma?

Anyone can be affected by scleroderma. Women are most commonly affected by this condition as compared to men. This disease is mostly seen in the age group of 30 to 50 years and is more common in adults than children. Multiple factors may lead to scleroderma, affecting people of all races and ethnic groups.


What Organs Are Affected by Scleroderma?

Scleroderma can lead to thickening or tightening and inflammation of connective tissue, such as collagen, all over the body. This can affect different parts of the body and cause scarring in different parts, including the lungs, heart, gastrointestinal system, and, most importantly, the kidneys.


Is There Any Latest Treatment for Scleroderma?

In a study conducted in Japan in September 2021, Rituximab was found to be a promising agent for treating scleroderma. The study was conducted as a double-blind, randomized, or placebo-controlled trial. And based on the results, Ritiximab was approved in Japan for treating scleroderma.


How Is Scleroderma Diagnosed?

The diagnosis of scleroderma is challenging as it involves multiple body systems. It can also be misdiagnosed as other conditions, such as rheumatoid arthritis or lupus. 
The diagnosis is made by conducting a physical examination of the patient followed by multiple tests, which may include -
- Blood tests.
- Pulmonary function tests.
- Electrocardiogram.
- Echocardiogram.
- Gastrointestinal tests.
- Kidney function tests.


What Food Is Best for Scleroderma?

Some of the food products that are recommended for scleroderma include -
- Fruits such as melon, grapes, berries, raisins, and cherries.
- Green leafy vegetables.
- Protein-rich foods include meat, fish, cheese, eggs, nuts, and beans.
- Milk and dairy products.


Is Homeopathy Helpful in Scleroderma?

Homeopathy can be considered another option in the management of scleroderma. But this treatment strategy is not considered a miracle cure for patients suffering from advanced stages of scleroderma. Homeopathy is also very useful in providing a supportive measure to control the disease's progression and may help relieve certain symptoms like pain.


What Is the Commonly Followed Treatment for Scleroderma?

The treatment of scleroderma involves multiple systemic approaches, in which the treatment is done based on the symptoms experienced by the patient. In the initial stage, treatments like phototherapy (light therapy) and certain medicines like Methotrexate and Cyclosporine that affect the immune system are done. In addition to that, the treatment focuses on treating symptoms such as inflammation, vascular issues, and tissue fibrosis.


What Type of Blood Test Is Done for Scleroderma?

The antinuclear antibody (ANA) blood test is considered one of the most important tests for detecting scleroderma and other connective tissue diseases. The elevated levels of these antibodies are found in more than 90 percent of the cases. The test is done by examining a blood sample under a fluorescent microscope.


How to Stop Scleroderma Progression?

Some of the following lifestyle changes can be made to control the progression of scleroderma which include -
- Staying active.
- Protecting the skin by using sunscreen.
- Avoiding foods that may cause heartburn.
- Stopping smoking.
- Stress management.

Last reviewed at:
18 Oct 2022  -  5 min read




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