What is Scleroderma?
Scleroderma is a rare autoimmune disease that causes the hardening of the patient’s skin and other associated connective tissues.
There are more cases of women affected by scleroderma than men. Most people diagnosed with scleroderma are between the ages of 30 and 50. Currently, there is no cure for scleroderma. However, there are many treatments available that can help to reduce the symptoms associated with scleroderma. Some types of treatment can also help to improve a patient’s overall quality of life as well.
There are several types of scleroderma. Sometimes, it may only affect the skin in patients. But at other times, scleroderma may also affect other body organs beyond the skin. Some examples of organs affected by scleroderma include:
- blood vessels
- internal organs
- the digestive tract
Signs and symptoms of scleroderma
Depending on the type of scleroderma you have, the signs and symptoms may vary according to the body parts that are affected in each case.
Most people with scleroderma have hardening or tightening of the skin. They may also experience a change in color of the skin in affected areas or in patches of skin.
The patches of skin are sometimes oval-shaped and discolored or appear in straight lines on the affected areas of the body, such as the hands, arms, legs, or chest. These patches can even appear on the face or lips of some affected patients with scleroderma.
The number of patches may vary depending on the person. The skin can sometimes appear shiny because of being pulled so tightly. Movement in the area affected may also become restricted because of the tightening of the skin.
Fingers and toes
An early warning sign that a person may have scleroderma is having symptoms present of Raynaud’s disease. Raynaud’s disease causes the blood vessels to contract in your fingers and toes. When a patient with Raynaud’s comes in contact with cold temperatures or in some cases emotional distress, their fingers or toes may turn blue. It also makes the person’s extremities feel numb and sometimes very painful. Raynaud’s disease, however, can also affect people who don’t have scleroderma as well.
The digestive system
The digestive system can be affected by scleroderma in many different ways. Scleroderma can cause heartburn in patients whose esophagus is affected. Early signs of scleroderma may also be seen in difficulty when swallowing.
Scleroderma can also cause a person to have cramps or stomach bloating. This can lead to constipation, diarrhea, or other digestive problems associated with the intestines as well.
Another early symptom caused by scleroderma is the problem of not being able to absorb nutrients within the digestive system.
Heart, lungs, and kidneys
Patients with scleroderma may also have difficulty with their heart, kidneys, or their lungs because of the disease. If left untreated, the severity of the disease in certain organs of the body can become life-threatening or even fatal.
Systemic scleroderma may cause inflammation, thickening, and tightening of connective tissues. This can have serious effects on a person’s kidneys.
Scleroderma renal crisis is a life-threatening problem that can affect people with systemic scleroderma. Sometimes high blood pressure and kidney failure can also occur if the problem is not addressed quickly,
Other symptoms of scleroderma may include:
- shiny and smooth skin, most common on your hands and face.
- Raynaud’s phenomenon
- Sores on your fingertips
- Red spots cover your chest and your face
- Painful or swollen joints
- Muscle stiffness and weakness
- Sjogren’s syndrome dry mouth or eyes
- Swelling of the hands and fingers
- Labored breathing or shortness of breath
- Belly cramps
- No apparent cause for sudden weight loss
Causes of Scleroderma
Scleroderma can sometimes be hard to diagnose because of all of its underlying factors that don’t always present themselves upon first visits to the doctor’s office.
People with scleroderma are also found to have high levels of collagen in their body’s tissues. Collagen is a protein that makes up the connective tissue in the human body. Collagen is present in a person’s skin tissue.
Doctors are uncertain why the abnormal collagen production begins in a person with scleroderma. They do, however, suspect that the body’s immune system plays a large role in it.
Another number of factors are also thought to cause scleroderma, including:
- Problems with the body’s immune system
- Environmental factors that may trigger the onset of the disease
Types of Scleroderma
There are several types of scleroderma that can affect the skin and body of otherwise seemingly healthy people. They include the following types:
Usually, this type of scleroderma only affects your skin. Morphea is a type of localized scleroderma, which comprises hard, oval-shaped patches that appear on your skin. They start out purple or red and then change to white in the middle. Usually, these patches appear on the hands, feet, upper chest, or face of the affected person. This kind of scleroderma can also attack a healthy person’s blood vessels and organs.
Linear scleroderma is another type of scleroderma that marks a person with lines of thickened skin, usually affecting the extremities or the face.
Systemic scleroderma, also known as generalized scleroderma, can affect many body parts and organs of the body. There are two types of systemic scleroderma which include:
A type of scleroderma that develops slowly. It involves the skin of your face, hands, or feet. This type of scleroderma can also cause damage to the lungs, the intestines, and the esophagus of the affected person.
A type of scleroderma that is named after five common symptoms associated with having this disease including:
- Calcium buildup
- Esophagus issues
This type of scleroderma has a sudden onset. The skin of your body, legs, arms, hands, and feet may become thickened and discolored. This form of scleroderma can also affect a person’s internal organs, including the kidneys, the heart, intestines, and digestive systems.
Diagnosis and treatment of Scleroderma
Diagnosis of Scleroderma
To diagnose a patient with scleroderma, your doctor will have to do an exam and ask several questions regarding the history of your health and that of your family’s genetics. The doctor may also perform several tests on you to rule out other diseases and causes, such as:
- Ct scans
- Imaging tests
- Breathing tests
- Blood tests
- Gastrointestinal tests
- Lung function tests
- Heart monitors
- Skin examination
- Skin biopsy
Treatment of Scleroderma
The treatment prescribed by a physician for scleroderma can greatly lower the complications associated with the disease. However, some severe complications of scleroderma may still occur even after treatment has been prescribed, including:
- High blood pressure
- Scar tissue of the face and skin
- Lung damage
- The ongoing loss of blood flow to extremities
- Inflammation of the muscles
- Organ failure
Some types of treatments prescribed by your doctor for the management of symptoms associated with scleroderma may include:
- Anti-inflammatory drugs like ibuprofen or aspirin may help with pain and swelling in hands, feet, and joint pain.
- Steroids may help with muscles, joints, and body problems.
- High blood pressure medication to steady your heart rate.
- Drugs prescribed may help to prevent scarring of the skin on the body and face.
- Procedures to open blood vessels in the lungs and arteries that are damaged.
- Antacids are prescribed for heartburn and indigestion problems.
- Medications, such as antibiotics for infections caused by scleroderma.
- Anti-diuretics are prescribed for diarrhea and bowel problems.
- Recommend an exercise routine to keep your body healthy and in good shape.
- Suggest including more fiber intake in your daily diet to help with the digestion of foods.
- Drink adequate amounts of fluids and water.
- Laser skin therapy to treat scarring caused by scleroderma.
- Physical therapy to help with joint and muscle damage.
- Recommend stress management to help you deal with your problems associated with the onset of scleroderma.
- Organ transplant in extreme cases of organ failure caused by the damage to organs as a result of scleroderma.
Famous people associated with Scleroderma
Born on May 17, 1956, Bob Saget was an American comedian and actor. His major television roles included Danny Tanner on the popular ABC TV series Full House, which aired from 1987 to 1995. He also starred in its Netflix sequel called Fuller House, which aired from 2016 to 2020. Other major roles of Bob Segat included How I Met Your Mother, a CBS sitcom that aired from 2005 to 2014, and he was the host of America’s Funniest Home Videos.
Saget was also a stand-up comedian, nominated for a Grammy Award for Best Comedy Album in 2014 for his album That’s What I’m Talkin’ About.
Bob Saget’s sister, Gay, had Scleroderma. She passed away three years after her initial diagnosis of the disease at 44.
After her tragic death, Bob dedicated his entire life to raising money for scleroderma research and to educate others about the debilitating disease. He also produced a movie about the life of his sister and the illness she battled with Scleroderma titled For Hope.
Bog Saget recently passed away suddenly on January 9, 2022, at the age of 65. The cause of his death is still under investigation.
Queen Latifah’s mother, Rita Owens, also had scleroderma. Diagnosed in 2013, she died in March 2018. Shee was also diagnosed with heart failure in 2004, which may have resulted from a case of undiagnosed scleroderma.
Dana Owens, aka Queen Latifah, is a rapper, actress, and producer who has always been supportive of scleroderma research since the onset of her mother’s disease, requiring her to use oxygen therapy for survival.
Actor Mark Teich also had scleroderma. Diagnosed in 1984, he was just 11-years old at the first onset of the disease.
“It came on quickly. I lost flexibility fast,” he stated. “I basically had to relearn everything, from how to write, and holding silverware to dressing myself.”
Mark Teich was from Detroit, Michigan. He was a popular comedian who starred in 4 gold CDs, a gold DVD, two Comedy Central Presents specials, and several television appearances, including The Disney Channel, Zeke and Luther, and The Suite Life on Deck. He also starred in “I’m in the Band,” and A.N.T Farm, among several other television shows and commercials that are too numerous to mention them all.
Although diagnosed with scleroderma many years earlier, Teich passed away from cancer in July 2021.
Another celebrity, Jason Alexander, doesn’t have scleroderma, but his sister had it. After watching her struggle with the disease, Alexander became an advocate for scleroderma. His sister, Karen, passed away in 2014 after a lengthy battle with scleroderma.
Born September 23, 1959, Jason Alexander was from Newark, New Jersey. His real name was Jason Scott Greenspan. An actor who played in the television series Seinfeld as the character George. Also starred in the movies The Hunchback of Notre Dame in 1996 and Pretty Woman in 1990. He is married to Daena E Title and has two children.
How you can help be an advocate for Scleroderma
The Scleroderma Society of Ontario is a registered charity established in 2007. The major goals of the organization are to:
- Increase awareness
- Promote patient wellness
- Support research in Ontario’s scleroderma community.
- Help people with scleroderma gain information about the disease
- Help find a cure!
- Develop patient education
- Bring patients together in peer-to-peer groups
- Grant research funding requests
- Facilitate external research funding
You, too, can help play a significant part in their work by increasing awareness of Scleroderma through participating in the organization’s campaigns and activities planned across Ontario.
3 out of 100,000 people develop systemic scleroderma every year. The average rate in Canada is approximately 40,000 individuals. That’s a significant number of sick people who need our help.
You can help make a tremendous difference by donating to the cause. Even a small donation will go towards the cause of supporting awareness, spreading education, patient advocacy, and research into such a rare disease.
By being an advocate, you can spread awareness about this debilitating disease and help bring about a positive change in the cure for scleroderma.
The Society of Ontario strives to ensure positive outcomes for every scleroderma patient. Heightening public awareness of the silent but deadly autoimmune disease is a step in the right direction.
Your small donation of money or time spent advocating goes a long way to making a big difference!