What is Scleroderma?

Scleroderma, which means “hard skin,” is an umbrella term for rare diseases that occur when the skin and connective tissues harden. Characterized by tightening patches of skin, this chronic disease cannot be cured nor transferred. Nevertheless, treatment is available to minimize damage. Also, it most often affects adult women ranging from 25-55 years of age.

Scleroderma has two classifications — systemic and localized. The difference between the two lies in the extent of their effects. While systemic scleroderma is more widespread as it affects the skin, blood vessels, muscles and even the internal organs, localized scleroderma generally affects just the skin and the muscle beneath it.

Localized Scleroderma

This is the skin-localized type of scleroderma, which is brought by excess in collagen deposits. As a result, the dermis and subcutaneous tissues thicken; hence, localized scleroderma forms. Some of its manifestations include fever, fatigue, joint pain, central nervous system involvement and disease in lymph nodes. It also causes laboratory abnormalities such as the creation of positive antinuclear bodies.

Although anyone can acquire this skin disease, Caucasians comprise most of the cases of this condition. In addition, it is more likely for women than men to develop localized scleroderma. Other factors that can affect the growth of this disease include trauma, infections, exposure to drugs or chemicals.

Effects of Localized Scleroderma

Even though localized scleroderma is not fatal, it can have serious negative effects on one’s life. Joint contractures affect the movement of the patient and, in rare instances, localized scleroderma can cause serious deformation of the face and other visible areas.

Systemic Scleroderma

Similar to localized scleroderma, systemic scleroderma comes as a result of increased collagen production. However, unlike the former, the effect of systemic sclerosis is not limited to the skin. It can affect one’s lungs, kidneys, blood vessels and other bodily organs. Alternatively referred to as diffuse scleroderma, it is common among adults of ages 30-50 years and women are more likely to be diagnosed than men. Other names for this condition are progressive systemic sclerosis and CREST (Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasia) syndrome.

The following are the various symptoms and complications of systemic scleroderma:

Calcinosis Scleroderma

Calcinosis is a medical condition brought by abnormal quantities of calcium salts within the soft tissues of the body. The size and shape of these deposits are varied and they are commonly stored in the fingertips. These deposits can also be present in many pressure areas like the extensor surfaces of the elbows, knees and hands.

In addition, it is also classified as a symptom of scleroderma. While plenty of pharmacological methods were tried, calcinosis scleroderma remains a disease without a definite cure.

Scleroderma Esophageal Dysmotility

The esophagus is the organ that helps transport food and liquids from the mouth to the different organs of the body. Esophageal dysmotility is the recurrent failure to deliver food and liquids because of spasmodic pain. Scleroderma esophageal dysmotility refers to the condition characterized by difficulty in swallowing or regurgitating food, heartburn and chest pain. Although it is allegedly an allergic reaction to food, this condition is also a symptom of scleroderma.

Scleroderma Renal Crisis

Scleroderma renal crisis (SRC) is a fatal complication of systemic scleroderma. And for every hundred scleroderma patients, an estimated 5 to 15 people develop this complication. Acute kidney failure and abrupt rise in blood pressure are some of its symptoms. Although SRC usually occurs to patients with an early and progressive condition, it can also act as the first symptom of scleroderma. In fact, 20 percent of SRC cases are reported among people who aren’t yet diagnosed with skin sclerosis.

Scleroderma Lung Disease

Systemic scleroderma is a skin condition, but it can deteriorate other parts of the body as well, such as the lungs. Scleroderma lung disease is the pulmonary effect of scleroderma. In fact, research estimates that 75 to 80 percent of people who have scleroderma may get afflicted by a pulmonary disease.

What is Morphea?

Morphea is a rare kind of localized scleroderma characterized by the discoloration of different parts of the skin. Specifically, patches of hardened, blemished skin can appear on one’s face, hands, torso, neck and other areas. In general, it only affects the skin. But on occasion, it can cause stiffness in the joints. Although relapse could occur, it usually resolves itself on its own. It is not contagious and its definite cause is still unknown. Commonly affecting school-aged children and adults in their 50s, this rare disease is identified as an immune disorder brought by overactive collagen-producing cells.

There are four subtypes of morphea — plaque, linear, pansclerotic and generalized. These are subdivided according to the depth of tissue involvement as well as their clinical presentation.

Plaque Morphea

Also called “plaque morphea”, this subtype is characterized by painless but itchy lesions. It is, in fact, the most common type of morphea and people who have this condition tend to have three to four oval patches of lesions.

Linear Morphea

Linear morphea is referred to as en coup de sabre, meaning “struck by the sword” because it features one band of thickened and discolored skin. It is most common on an arm or leg. If the lesion extends beneath the skin, deformities may occur in different areas of the body, such as teeth alignment, eyesight problems and muscle and bone issues. Patients of this sub type are usually younger children.

Pansclerotic Morphea

This sub type of morphea is characterized by widespread plaques, covering almost the entire body. Hands and feet are the only skin parts spared. It is also rapidly aggressive. Because of this, pansclerotic morphea is the type that needs immediate strong treatment the most.

Symptoms of Scleroderma

Unlike other illnesses dubbed as silent killers because of invisible symptoms, scleroderma can be easily diagnosed because of its noticeable manifestation on the body. However, it is best to see a doctor before assuming you have this disease. Also note that symptoms may vary depending on the type of scleroderma.

Thick Skin on the Face

Hardening of the skin is likely to occur on one’s hands, face and arms. However, there are instances in which the trunk and leg skin thickens as well.

Swollen Fingers from Cold

Puffiness of hands and feet may occur especially in the early morning. Bending the fingers may become difficult. This would make it hard for the patient to perform simple day-to-day activities.

Glossy Skin

Another effect of scleroderma is shiny or waxy skin. This usually occurs on the fingers and toes of the patient.

Freezing Hands

Due to decreased blood flow to the fingers and toes, scleroderma patients are prone to cold intolerance. Feeling tingly and numb is common, especially with the hand areas.

Bruises with a Hard, White Center

Due to calcinosis, small, white, calcium-filled lumps may form in or beneath the skin. On occasion, these lumps may break through the skin and ooze a chalky material. This can also cause an infection.

White, Chalky Lumps

Another symptom of scleroderma is the presence of white, chalky lumps caused by increased calcium in the body. This is not due to overconsumption of calcium. Raynaud’s disease, which causes calcium deposits in the lungs and on skin, causes this symptom.

Other Symptoms

Other symptoms of scleroderma include elderly skin rashes and leathery skin. Kidney, lung, stomach and other organ issues may also develop with the onset of scleroderma.

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Causes of Scleroderma

Although doctors have not yet found the definite cause of scleroderma, they have found several conditions and factors that may cause it. Among them is fibrosis or the thickening of the skin. Fibrosis occurs due to the overproduction of connective tissues. This is why people sometimes consider scleroderma as an autoimmune disorder.

Although the condition is not contagious, some researchers claim that environmental factors play a role in the development of this disease. Medical professionals have also associated scleroderma with lupus, an autoimmune disorder characterized by inflammation, because of the overlapping symptoms of the two diseases. Since experts think of lupus as hereditary, they also consider genetic factors as a possible cause of scleroderma.

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Living with Scleroderma 

The impact of the rare autoimmune disease differs depending on what type of disease the patient acquired. In fact, localized and systemic scleroderma cause differing levels of a handicap to the patient.

For people diagnosed with localized scleroderma, they can temporarily relieve irritating, aching, patches of marred skin through certain creams. However, the physical effects of this condition may still impact the psychological well-being — and possibly the social status — of the patient.

On the other hand, systemic scleroderma is more complicated since one of its common effects is organ failure. The deterioration of the body begins first with calcinosis. Calcium deposits in the connective tissues lead to the thickening of the skin. At this stage, patches of white spots and swollen joints or fingers occur. Then, whitish or bluish feet and hands occur as a response to cold and anxiety. This is called Raynaud’s phenomenon. Along with this, esophageal dysfunction or swallowing difficulty occurs. Then, sclerodactyly brings about the thickening and tightening of the skin on the fingers. This is due to excessive collagen deposits. Lastly, small red spots on the hands and face characterize telangiectasia due to the inflammation of the blood vessels.

Death by scleroderma can occur because of complications and organ failures such as pulmonary hypertension, pulmonary fibrosis and scleroderma renal crisis. People with systemic scleroderma are more likely to die than those with localized subtype.

Treatment for Scleroderma

Presently, there is no cure for scleroderma. However, treatment options are available to mitigate the suffering and prevention of further complications with this autoimmune disease.

For vitiligo, or the white patches in the skin, moisturizers or corticosteroid creams can be applied. However, the patches sometimes go away on their own. Either way, there is no certified method in removing the spots.

For joint pain, oral medication prescribed by the doctor such as corticosteroid pills could help in reducing the inflammation. Nonsteroidal anti-inflammatory drugs (NSAIDs) like aspirin, naproxen and ibuprofen may help in alleviating the symptoms, too. Vasodilators, a medicine that aims to relax the blood vessels, may also aid patients whose cold intolerance causes their feet and hands to ache. In order to control blood pressure, doctors may prescribe an angiotensin-converting enzyme (ACE) inhibitor. Your doctor may also prescribe a special diet in order to lower stomach acidity.

Supplements for Scleroderma

Some supplements can alleviate the effects of scleroderma. Here are some of them:

EDTA Calcium Disodium Powder

EDTA calcium disodium is a soluble, crystalline, white powder which can alleviate the symptoms of scleroderma by reducing the calcium level of the body. Take 600 mg (scant 1/16 tsp) of this dietary supplement per day with meals, or as directed by a physician.

PABA Powder

Para-AminoBenzoic Acid (PABA) is a type of non-protein amino acid that possesses anti-fibrosis effects through increasing oxygen consumption by tissues. Take 100 to 500 mg of this supplement per day, which is equivalent to 1/16 to 1/4 tsp.

Curcumin 95% Natural Turmeric Extract Powder

Curcumin can help in the detoxification process because it can induce apoptosis in scleroderma lung fibroblasts. Roughly 1,000 mg for this product is equal to less than 1/2 tsp, or a little more than 3/8 tsp.

Vitamin E Softgels

A lowered antioxidant level is an effect of scleroderma. Taking vitamin E as a supplement is a must in order to boost this deficiency. As a dietary supplement, take 1 softgel daily with a meal, or as otherwise directed by a doctor.

The Bottom Line

Scleroderma is more than just a skin condition. Localized scleroderma can cause deformation to the bones and even teeth misalignment. Systemic scleroderma is more fatal because it can lead to other organ illnesses. The research regarding this rare autoimmune disease is still exploratory and experts haven’t found a standard cure yet. However, doctors offer treatment options to help alleviate the stress brought by this condition. Since environmental factors play a role in the growth of this autoimmune disease, adopting a healthy lifestyle and taking the proper supplements are a few ways of preventing it.