SCLERODERMA: MORE THAN JUST A SKIN DISEASE

Scleroderma is an autoimmune disease that leads to the hardening and tightening of the skin. It can also cause damage to the blood vessels, the digestive tract, and internal organs like the lungs, heart, and kidneys. The reported prevalence of Scleroderma varies significantly in different countries, with a higher incidence observed in Europe and the Americas than in East Asia. Studies pertaining to prevalence based on ethnicity indicate a higher frequency among blacks. Furthermore, women have shown to be at 4 to 9 times higher risk than men.

Because there isn’t yet a definitive test for scleroderma, doctors rely on clinical symptoms and medical history to make a diagnosis. The symptoms of scleroderma can include Raynaud’s phenomenon (blue fingers in cold weather), dilated blood vessels in the skin (spider veins), hardened thickened skin, and heartburn.

Scientists still don’t know what causes scleroderma and there still isn’t any cure for this chronic systemic disease. Nevertheless, patients living with scleroderma use certain coping strategies in addition to medications that can help manage its symptoms.

Many celebrities including Robin Williams, Queen Latifah, Bob Saget, and Dana Delany have supported further research on scleroderma and raised funds and awareness.

What Is Scleroderma?

Scleroderma, sometimes known as systemic sclerosis, is a chronic autoimmune disease that affects around 2.5 million people worldwide. It causes parts of the skin to become hard and thick and leads to scarring and inflammation in several organs including the heart, lungs, and intestines. 

The immune system of people with scleroderma triggers inflammation in the skin and other connective tissue in the body. This autoimmune reaction causes the body to produce excessive amounts of collagen (a protein naturally produced in the skin and connective tissue).

The resulting collagen buildup causes the skin to become hard and tight resulting in scleroderma.

Scleroderma does not only cause cosmetic problems. Chronic inflammation and excess collagen buildup in soft tissue can damage blood vessels and the digestive tract. They can also cause the scarring of organs like the lungs and heart.

Scleroderma is a relatively rare disease, but it can affect anyone. Despite the research dedicated to this rare skin and systemic disease, no cure has yet been found.

What Are The Types of Scleroderma

Scleroderma can be localized and only affect the skin, or it can be systemic and affect major organs in the body.

Localized Scleroderma

Localized scleroderma involves only the skin and its underlying connective tissue. The localized type of scleroderma can appear in two forms:

Morphea

In morphea, the changes to the skin appear as colorless, painless round skin patches. Patients usually find these skin patches on their back, belly, or chest. But some also have them on their arms, legs, or face to a lesser extent. Morphea is the more common type of localized scleroderma.

Linear

In linear scleroderma, the thickening of the skin occurs in the shape of a band or a thick line. This type of skin thickening appears mostly on the face (most commonly the forehead) and extremities. Linear scleroderma is the most common type of scleroderma in children.

Both types of localized scleroderma are restricted to the skin and its underlying tissue. They do not affect any major organs in the body, so localized scleroderma is considered the less severe type of scleroderma.

Systemic Scleroderma

In addition to the skin, systemic sclerosis also affects the blood vessels and major internal organs in the body. Systemic scleroderma is the more dangerous type of scleroderma. Recently, the passing of beloved comedian and champion of scleroderma research, Bob Saget, brought attention to this form of scleroderma since the late actor’s sister Gay had succumbed to this disease. Systemic Scleroderma can be divided into two types:

Limited

Limited systemic scleroderma, previously known as CREST syndrome, starts happening gradually and is typically limited to the skin on the fingers and toes. People with limited sclerosis find it hard to make a fist because the skin on their fingers becomes dry and rough.

The skin damage in limited systemic sclerosis could also affect the skin on the face, forearms, hands, and below the knees.

Limited scleroderma is less severe than diffuse scleroderma, but it can get worse with time and eventually affect the digestive system and the lungs.

Diffuse

30 to 40% of all scleroderma patients suffer from diffuse systemic scleroderma which manifests more suddenly than limited scleroderma.

In systemic sclerosis, the skin hardening in the fingers and toes starts spreading rapidly to the rest of your body reaching above the elbows and knees. This spread of sclerosis can reach and damage the lungs, kidneys, and heart.

What Causes Scleroderma?

It’s still uncertain how someone gets systemic sclerosis or what triggers the reaction of the immune system. However, there are several factors that seem to increase the risk of getting scleroderma:

• Gender: Scleroderma is much more common in women than in men.
• Genetics: You are more likely to have scleroderma if you’re a relative of a scleroderma patient.
• Age: Scleroderma is most common among adults between the ages of 30 and 50.
• Environmental factors: Exposure to harmful substances, like silica and epoxy resins, can increase your risk of systemic sclerosis.

What Are The Symptoms of Scleroderma

Although all types of scleroderma cause changes in the skin, patients can have other symptoms depending on the type of scleroderma they have:

Raynaud’s Phenomenon

Raynaud’s phenomenon (RP) is the most common symptom of systemic sclerosis.

Raynaud’s disease happens because the blood vessels in the fingers become restricted which limits the blood flow to the fingers. As a result, the fingers turn white, red, or blue and feel numb in response to cold or stress.

Skin Symptoms

Hard, shiny skin is a common symptom for almost everyone with scleroderma. Other skin-related symptoms of scleroderma include:

• Swelling and itchiness
• Skin color changes (can become lighter or darker)
• Red spots (telangiectasia) on the face and hands
• Bumps under the skin (especially under fingertips) because of calcium deposits

These symptoms most commonly show on the face, feet, fingers, and hands. But they can also appear on the skin of the upper arms, forearms, lower legs, thighs, and abdomen.

Heart and Lung Problems

Scleroderma can damage the blood vessels of the heart and lungs and cause scarring of their tissue.

The restriction of pulmonary blood vessels can lead to pulmonary hypertension (high blood pressure in the arteries of the lungs). Additionally, scarring of the lung tissue causes breathing difficulties. In 2018, famed hip-hop artist and actor Queen Latifah lost her mother to scleroderma-related interstitial lung disease (ILD) that caused scarring on her lungs, leading to breathing problems.

Scleroderma can also cause arrhythmia (irregular heartbeats) because of the damage to the heart tissue.

Digestive Symptoms

Some people experience digestive complications associated with scleroderma. These digestive problems can cause the following symptoms:

• Heartburn
• Constipation / Diarrhea
• Difficulty with swallowing
• Abdominal cramps

What Are Other Complications of Scleroderma?

Scleroderma can lead to complications affecting other areas of the body such as:

• The teeth: When the skin on the face becomes too tight, it becomes harder to open the mouth. This makes it difficult to brush your teeth causing dental problems after a while.
• Kidneys: The damage in the blood vessels can increase the blood pressure in the kidneys, consequently leading to kidney failure.
• Joints: The skin can become too tight that it becomes hard to flex your joints. Restricted joint movement can limit their flexibility and create joint pain from scleroderma.

How Can You Treat Scleroderma?

To this day, no treatment has been found that can cure scleroderma. In spite of that, some drugs can relieve or lessen some of the symptoms of scleroderma:

• Antacids for heartburn.
• Calcium channel blockers improve blood circulation and treat Raynaud’s phenomenon.
• Blood pressure medication (ACE inhibitors) can reduce hypertension and treat kidney or pulmonary diseases.
• Anti-inflammatory drugs to relieve joint and muscle pain.
• Immunosuppressants can reduce the overreaction of the immune system.

Although you cannot cure your scleroderma, you can minimize its impact on your life. Regularly moisturizing the affected skin, undergoing physiotherapy to loosen tight skin, and keeping your hands and feet warm can all make living with scleroderma easier.

FAQs

What is the survival rate for scleroderma?

The life expectancy of someone with scleroderma depends on the type of scleroderma they have. Localized scleroderma is not life-threatening, and patients can live an otherwise normal healthy life. On the other hand, patients with severe systemic scleroderma live on average 3 to 15 years depending on the health complications they develop due to the disease.

What is the difference between CREST syndrome and scleroderma?

CREST is one type of scleroderma, called “limited scleroderma”, that only affects the skin of the face, arms, and legs. CREST syndrome does not affect any major organs like diffuse scleroderma.

What celebrities had scleroderma?

The mother of actress and artist Queen Latifah died of scleroderma in 2018. Comedian Bob Saget’s sister also died of systemic sclerosis back in 1994, and the late actor spent years advocating finding a cure for scleroderma. Actor Jason Alexander’s sister Karen passed away in 2014 due to the disease. Diagnosed with scleroderma at the age of 11, actor and comedian Mark Teich survived the disease, eventually succumbing to cancer aged 48. The sister of Football Hall of Famer Barry Sanders died from scleroderma at 26 years of age.

What are the first signs of scleroderma?

Limited scleroderma often starts with the fingers turning white, red, or blue with the cold (Raynaud’s phenomenon). The more severe systemic sclerosis can happen suddenly causing renal, cardiac, and pulmonary problems.

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Sources:

• Scleroderma - Symptoms and causes - Mayo Clinic
• Scleroderma - StatPearls - NCBI Bookshelf
• Scleroderma - NHS
• Scleroderma - Medscape

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