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Scleroderma

Foothill Dermatology Medical Center Blog Scleroderma

Generalized morphea: This type of scleroderma causes widespread patches of hard, thickened skin.

What is scleroderma?

When a person has scleroderma (sclare-oh-dur-muh), the body makes too much collagen. This excess collagen, the substance that holds our body together, causes hardening and tightening.

Most people have hardening and tightening on their skin. Scleroderma means “hard skin.” But this disease can affect more than the skin. Joints, muscles, and even internal organs like the kidneys and lungs can harden and tighten.

Many people who have scleroderma lead normal – or almost normal – lives.

Where you have the hardening and tightening varies with the type of scleroderma you have. When scleroderma affects the skin and sometimes the underlying tissue, a person has localized scleroderma.

There are many types of localized scleroderma, including:


Morphea on skin		 Morphea (more-fee-uh): People see 1 or a few patches of thickened skin, which are usually red or purple. The patches can itch but are usually painless. Sometimes the excess collagen develops deep in the skin. In rare cases, morphea affects muscle.

Patches of morphea on patient's back		 Generalized morphea: Patches of morphea can develop on different areas of the body. The patches can grow together. Some people see a band of thickening skin on an arm or leg. This type can go deep into the tissue beneath the skin.

Linear morphea on lower leg and foot		 Linear scleroderma: Often beginning in childhood or the teenage years, this type causes a line of thickening skin, usually on an arm or leg. The hardening can extend beyond the skin, going deep into to the muscle and sometimes the bone.

En coup de sabre on forehead		 En coup de sabre: A line of thickened skin forms on the scalp, face, or both, and the tissue beneath disappears.
A person with a mild case develops a bit of tissue loss. En coup de sabre can also be disfiguring.

A dermatologist can use a filler, such as hyaluronic acid, to restore the face.

When scleroderma affects an internal organ, the person has a type of scleroderma called systemic sclerosis. There are 2 types of systemic sclerosis:


Sores and calcium deposits on fingers		 Limited cutaneous scleroderma: This often develops slowly. Hard, thick, and tight skin usually forms below the elbows and knees and sometimes on the face and neck.
Many people develop calcium deposits beneath their skin and sores, as show here.

The hardening can also occur in the digestive tract and internal organs like the lungs. The health problems caused by this type are less serious than those caused by diffuse cutaneous scleroderma.

Woman is unable to straighten her fingers		 Diffuse cutaneous scleroderma: In just a few weeks or months, hard, thickened skin can develop on many areas of the body. The excess collagen also develops on one or more internal organ.

The hardening can cause a person to lose the ability to move freely. The patient shown in this picture lost the ability to straighten her fingers.

Under the care of a team of doctors who specialize in different areas of the body, it may be possible to manage this type.

There is no cure for scleroderma, but treatment can help reduce the effects of this disease.

Because scleroderma can affect the body in so many ways, many people who have scleroderma receive treatment from a team of medical specialists. A dermatologist may be a part of this team and can treat problems with the skin.

Images used with permission of Journal of the American Academy of Dermatology:

References
Denton, CP and Black CM. “Scleroderma (systemic sclerosis).” In: Wolff K, Goldsmith LA, et al. Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008:1553-62.

Falanga V and Killoran CE. “Morphea.” In: Wolff K, Goldsmith LA, et al. Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008:543-6.

Fett N and Werth VP. “Update on morphea: Part I. Epidemiology, clinical presentation, and pathogenesis.” J Am Acad Dermatol 2011;64(2):217-28.

Fett N and Werth VP. “Update on morphea: Part II. Outcome measures and treatment. J Am Acad Dermatol 2011;64:231-42.

Rőcken M and Ghoreschi K. “Morphea and lichen sclerosus.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:1469-76.

Walls A, Goldberg D, et al. “Correction of morphea en coup de sabre with hyaluronic acid filler.” Presented as a poster at the 2012 Annual Meeting of the American Academy of Dermatology and published in the J Am Acad Dermatol. 2012:66(4) AB209. No commercial support identified.

Zwischenberger BA and Jacobe HT. “A systematic review of morphea treatments and therapeutic algorithm.” J Am Acad Dermatol 2011;65(5):925-41.

Scleroderma has many signs and symptoms. The following images let you see some of the ways it can affect your skin.

Hard, thickening, or tight skin: This trait is what gives scleroderma its name. Some people develop 1 or 2 patches of hard, thick skin. Others have widespread patches on their body. The hard, thick skin can feel anchored in place. If you have morphea (more-fee-uh), the most common type of scleroderma, the patches may not feel too hard. In time, the hardened skin may soften.

Hair loss and less sweating: Where you have hardened skin, you’ll often see that the area is shiny, discolored, and has hair loss. The hardened skin also tends to lose its ability to sweat.

Dry skin and itch: Scleroderma causes extremely dry skin, and dry skin itches. The extreme dryness can cause the skin to breakdown and sores can form.

Skin color changes: The patches of hardened skin can be lighter or darker than your natural skin color. Some people develop violet-colored skin, which means that the scleroderma is active and expanding. This patient has darker and lighter (white) areas that are hard to the touch.

Source: JAAD, “Borrelia-associated early-onset morphea” Prinz, Feb. 2009.

Salt-and-pepper look to the skin: This tends to develop on the upper back, chest, or scalp (along the hairline). The skin may feel hard or tight — but not always. If you have a salt-and-pepper look on your skin, you should see a doctor. This can be a sign that you have a type of scleroderma that affects internal organs. The sooner you are diagnosed and treated, the better your prognosis (what is likely to happen).

Stiff joints and difficulty moving them: When the hard, thickening, or tight skin forms over a joint (i.e., the jaw, wrist, or finger), the tightness can make it difficult to move that joint. The patient in this picture cannot fully open her hands because of tight skin. Physical therapy can help you keep the full range of motion. Without it, you may lose the ability to fully straighten or bend a finger, elbow, wrist, or other part of your body.

Muscle shortening and weakness: The hardening and tightening sometimes extends to the muscle. This can cause the muscle to weaken and shorten. You may be unable to stretch the muscle. If you have muscle weakness, it’s important to tell your doctor so that the cause can be found and treated.

Loss of tissue beneath the skin: This girl has a type of scleroderma called Parry-Romberg syndrome (PRS), which can cause loss of muscle, cartilage, and bone. PRS usually affects one side of the face as shown here. It can also affect an arm, leg, or trunk. PRS is very rare. Other forms of scleroderma can also cause loss of tissue beneath the skin.

Source: JAAD, A review of Parry-Romberg syndrome, October 2012

Bone may not grow as it should: When a child gets some types of scleroderma, such as linear scleroderma or en coup de sabre, the scleroderma can interfere with bone growth. A leg may not develop as it should. If scleroderma affects the head, the face may become deformed. These deformities are rare.

Source: JAAD, Pediatric morphea. Sep 2008

Sores and pitted scars on the fingers: When a person has a type of scleroderma that also affects the internal organs, it’s common to have skin sores. These sores tend to develop on skin that is tightly stretched. Sores are especially common on the fingers. Some people develop pinhead-sized pitted scars on their fingertips and the sides of their fingers.

Source: JAAD, Chung, May 2006, A pilot trial of treprostinil for the treatment and prevention of digital ulcers in patients with systemic sclerosis

Calcium deposits beneath the skin: Called calcinosis (KAL-sin-OH-sis), this occurs in the connective tissue beneath the skin. You may feel 1 or more hard, painful lumps beneath your skin. If a calcium deposit breaks through the skin, it can be very painful and you’ll see a white or yellow chalky substance. An infection and painful open sores can develop.

Source: JAAD article, Calcinosis cutis, Reiter, July 2011

Visible blood vessels: This happens when tiny blood vessels near the surface of the skin swell. You may see tiny red spots, usually on the hands and face. It is not painful, but many people dislike the way these look. Treatment is available.

Extreme sensitivity to cold, stress, or both: This is often an early warning that scleroderma affects the internal organs.

The medical name for this symptom is Raynaud’s phenomenon. It causes certain parts of your body, usually the fingers, toes, ears, or tip of your nose to feel cold and go numb in cold temperatures or when you feel stressed. You skin may turn white and then blue. As the blood flow returns to normal, the affected areas often turn red.

Not everyone with Raynaud’s has scleroderma. The restricted blood flow, however, can cause problems for anyone with scleroderma. Raynaud’s can damage the skin on the fingers, causing sore or pits.

When scleroderma affects internal organs
Some types of scleroderma affect the skin and internal organs like the lungs and kidneys. The following symptoms can be a warning sign that scleroderma is affecting an internal organ:

Digestive system

Other organs

If you have any of these, you should see a dermatologist, rheumatologist, or other doctor who treats scleroderma. The sooner you are diagnosed and treated, the better your outcome.

Images
Images 1,5, and 12 used with permission of the American Academy of Dermatology National Library of Dermatologic Teaching Slides.

Images used with permission of Journal of the American Academy of Dermatology:

References
Denton, CP and Black CM. “Scleroderma (systemic sclerosis).” In: Wolff K, Goldsmith LA, et al. Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008:1553-62.

Falanga V and Killoran CE. “Morphea.” In: Wolff K, Goldsmith LA, et al. Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008:543-6.

Fett N and Werth VP. “Update on morphea: Part I. Epidemiology, clinical presentation, and pathogenesis.” J Am Acad Dermatol 2011;64(2):217-28.

Rőcken M and Ghoreschi K. “Morphea and lichen sclerosus.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:1469-76.

Singh A, S Ambujam S, et al. “Salt-and-pepper appearance: A cutaneous clue for the diagnosis of systemic sclerosis.” Indian J Dermatol. 2012 Sep-Oct; 57(5): 412–413.

Who gets scleroderma?

Scleroderma is rare. The Scleroderma Foundation estimates that about 300,000 people in the United States have some type of scleroderma.

The following explains who has a higher risk of developing scleroderma.

Gender: Women are more likely than men to get most types of scleroderma. One type, linear morphea, occurs about equally in men and women.

Age: This disease can occur at any age. Children and seniors get scleroderma. One type, linear morphea, tends to develop before 18 years of age. The other types are most likely to begin between the ages of 30 and 50.

Race: Whites and Asians are most likely to get the types of scleroderma that affect only the skin and the tissue that lies beneath.

The Choctaw Native Americans in southeastern Oklahoma seem to have the highest risk of developing scleroderma that affects the skin and internal organs.

Several studies have shown that African Americans also have a higher risk of developing scleroderma that affects the skin and internal organs. In the United States, about 70% of people who get this type are African American.

What causes scleroderma?

Researchers are looking into what actually causes scleroderma. We do know that this group of rare diseases is:

We also know that a person develops scleroderma when the body makes too much collagen. This excess collagen is what causes the skin to thicken and harden. It can also cause hardening and thickening in the tendons, joints, and parts of the internal organs.

Why this happens is still a bit of a mystery. It’s believed that something in the person’s immune system goes wrong, causing the body to make too much collagen.

It’s also possible that something in the person’s environment could trigger scleroderma.

As far back as 1914, we’ve noticed that scleroderma is more common in people exposed to silica dust. Those exposed to silica dust on the job include miners, foundry workers, and roofers. More research is needed, however, to know whether silica dust increases the risk.

We also know that children who develop morphea, the most common type of scleroderma, are more likely to have a blood relative who has morphea. Genes may play a role.

References
Denton, CP and Black CM. “Scleroderma (systemic sclerosis).” In: Wolff K, Goldsmith LA, et al. Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008:1553-62.

Falanga V and Killoran CE. “Morphea.” In: Wolff K, Goldsmith LA, et al. Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008:543-6.

Fett N and Werth VP. “Update on morphea Part I. Epidemiology, clinical presentation, and pathogenesis.” J Am Acad Dermatol 2011;64(2):217-28.

Fett N and Werth VP. “Update on morphea: Part II. Outcome measures and treatment. J Am Acad Dermatol 2011;64(2):231-42.

Marie I, Menard JF, et al. “Association of occupational exposure with features of systemic sclerosis.” J Am Acad Dermatol. 2015;72(3):456-64.

Martin JR, Griffin M, “Systemic sclerosis (scleroderma) in two iron ore mines.” Occup. Mod. 1999;49:161-9.

McKinley-Grant L, Warnick M, et al. “Cutaneous manifestations of systemic disease.” In: Kelly AP and Taylor SC. Dermatology for skin of color. McGraw-Hill Companies, China, 2009:495-7.

Rőcken M and Ghoreschi K. “Morphea and lichen sclerosus.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:1469-76.

Scleroderma Foundation. “What is scleroderma?” Website last accessed February 2016.

How is scleroderma diagnosed?

If you have scleroderma, it can take time to get the diagnosis. Scleroderma is rare, and the signs and symptoms are similar to many other diseases.

Doctors who most commonly diagnose scleroderma are dermatologists and rheumatologists. Dermatologists have expertise in diagnosing diseases that affect the skin, and rheumatologists specialize in diseases that affect the joints, muscles, and bones.

To diagnose scleroderma, a doctor usually begins by asking you about your symptoms, health, and medical history. The doctor will also examine your skin for signs of hardening and thickening.

If you have hard, thickened skin, a dermatologist may perform a skin biopsy to help diagnose you. This is a fast-and-easy test that your dermatologist can perform during an office visit. Your dermatologist will remove a bit of affected skin so that it can be examined under a microscope.

While a skin biopsy can be helpful, no medical test can tell whether you have scleroderma. Doctors use this test and others to help them find signs that you have scleroderma. Other tests that can help a doctor diagnose scleroderma include blood tests, x-rays, a test to measure how well your lungs are working, and a CAT scan.

A blood test can tell your doctor whether you have something in your blood called “elevated antinuclear antibodies.” About 95% of people who have scleroderma have this, but people with other diseases also have this.

There is currently no cure for scleroderma, but treatment can improve a person's quality of life.

Treatment works best when started early

If the diagnosis is scleroderma, treatment is most effective when started shortly after the disease has begun. At that time, physical and occupational therapy can help you keep your ability to straighten and bend your joints and maintain your daily life.

Started early, treatments like phototherapy (light therapy) and medicines that work on the immune system like methotrexate and cyclosporine can help diminish scleroderma.

Working with a physical therapist can help you:

Your dermatologist may refer you for physical therapy.

How do dermatologists treat scleroderma?

A dermatologist can treat skin problems like hardened skin and visible blood vessels.

If you have hard, thickening skin on a joint (jaw, fingers, elbow), treating the skin early can help you keep your ability to open and close your mouth or bend and straighten your fingers.

Before treating you, your dermatologist will want to know how deeply the hardening reaches into your skin and the tissue beneath. Sometimes, it’s easy to tell how far the hardening reaches. At other times, a patient needs an MRI (magnetic resonance imaging).

The type of treatment you receive will depend on how deeply the scleroderma goes — and many other factors, including the type of scleroderma you have and what skin problems the scleroderma is causing.

The U.S. Food and Drug Administration (FDA) has not approved any drug to treat scleroderma on the skin.

Still, there are effective treatments to treat the skin problems. These include:

Swelling and patches of hard-feeling skin
If you have only a few patches of morphea (a type of scleroderma) on your skin, the following medicines can be effective:

Itch: To treat this, your dermatologist may recommend moisturizer, camphor, or menthol.

Dry skin: A moisturizer can help heal the dry skin.

Calcium deposits beneath the skin: Soaking in warm water can help reduce these. A strong corticosteroid like prednisone can treat large calcium deposits that develop beneath the skin. Laser treatment can also be helpful.

Morphea on the top layers of your skin: A type of light treatment called narrowband UVB treatment can be helpful.

When the morphea goes deeper, UVA-1 phototherapy may be prescribed. UVA-1 produces long wavelengths that can deeply penetrate the skin to reduce hardness. It can also treat the itch. Some patients see complete clearing of their skin.

Areas of darkened skin: Some patients who have morphea develop discolored skin. The discoloration can go so deep that bleaching does not work. Intense pulsed light (IPL) treatments help some patients.

Skin hardening in patients who have a lot of scleroderma on their skin: A type of phototherapy called PUVA can reduce this. When using this type of phototherapy, you take a medicine called psoralen before getting UVA treatment.

Laser treatments can reduce and sometimes eliminate visible blood vessels. Your dermatologist may treat you in the office or refer you to a dermatologist who specializes in laser therapy.

Visible blood vessels: Laser therapy can help get rid of or reduce these.

Hardness reaches beyond your skin into the tissue below: Medicines that reduces the inflammation or stops the immune system from being so reactive can be effective. These include:

In some cases, a dermatologist may prescribe more than one of these medicines. One small study found that when morphea covers a large part of the body, appears on the face, or covers a joint, it can be helpful to receive all of these treatments:

Severe scleroderma that affects the skin and internal organs: For this, a dermatologist may recommend extracorporeal photopheresis (ECP).

A study found that this treatment can be effective for severe scleroderma when ECP is started early. In this study, researchers found that ECP could stop the disease from worsening and reduce problems with the skin.

ECP helps to destroy diseased cells.

Getting ECP takes to 2 days. It begins with a blood draw. The white blood cells are removed from the blood, and the drawn blood is then returned to the patient.

The white blood cells are treated with psoralen (a medicine that makes cells more sensitive to UV light) and then exposed to UVA light.

This treatment helps destroy diseased blood cells. The patient’s treated white blood cells are then injected into the patient.

Side effects are possible with the different treatments described here. It’s important to talk with your doctor so that you know the possible side effects and ones that you may have a higher risk of developing.


Outcome: How can treating the skin help a patient who has scleroderma?

While the hardened skin often clears on its own in 3 to 5 years, scleroderma can cause irreversible changes.

Darkened skin and light patches can be permanent. Without early treatment, scleroderma can destroy tissue beneath your skin. This can leave you with an area that has less tissue under the skin.

Treating the skin can prevent these changes. It can also reduce how much scleroderma you get on your skin, which can prevent skin hardening over joints. Once you lose the ability to move a joint, this loss of movement is often permanent — even when the skin softens.

Treating your skin can also improve your quality of life. Most people feel better when they have less hardening and fewer signs or scleroderma on their skin.

Images
Images 1 and 4: Thinkstock
Image 2: Image used with permission of the American Academy of Dermatology National Library of Dermatologic Teaching Slides.
Image 3: J Am Acad Dermatol. 2005;53(2):S115-9.

References
Das S, Bernstein I, et al. “Correlates of self-reported quality of life in adults and children with morphea.” J Am Acad Dermatol 2014;70(5):904-10.

Fett NM. “Morphea (localized scleroderma).” JAMA Dermatol. 2013;149(9):1124.

Fett NM and Werth VP. “Update on morphea Part II. Outcome measures and treatment.” J Am Acad Dermatol 2011;64(2):231-42.

Gordon Spratt EA, Gorcey LV, et al. “Phototherapy, photodynamic therapy and photophoresis in the treatment of connective-tissue diseases: a review.” Br J Dermatol. 2015;173(1):19-30.

Krasagakis K, Dippel E, et al. “Management of severe scleroderma with long-term extracorporeal photopheresis.” Dermatology. 1998;196(3):309-15.

Zwischenberger BA and Jacobe HT. “A systematic review of morphea treatments and therapeutic algorithm.” J Am Acad Dermatol 2011;65(5):925-41.

10 ways to relieve discomfort

Applying medicine: Following a dermatologist’s treatment plan may reduce skin changes and discomfort.

People get different types of scleroderma, and there are many signs and symptoms.

While signs and symptoms vary from person to person, most people develop skin problems at some point. Hardening and thickening skin is common. Dry skin, itch, discolored skin, and visible blood vessels are also possible.

Dermatologists recommend the following when scleroderma affects the skin:

  1. If you have skin problems, see a dermatologist who has experience treating scleroderma. Treatment for your skin can help reduce signs and symptoms, helping you to feel more comfortable.
  1. Make time to treat your skin. Having scleroderma can feel overwhelming. If you are also treating other symptoms, it can feel less important to treat the skin. Treating the skin is important. Started early, treatment can help you maintain your ability to move when hardening skin develops over a joint like a finger, knee, or jaw.
  1. If a physical therapist has given you exercises to do at home, do them! This can prevent or slow down loss of movement. These exercises can also help increase blood supply to your skin, which can heal with healing.
  1. Keep warm any area with thickened skin and sores. When the skin thickens and sores develop, it leads to poor circulation in that area. Keeping this area warm can increase blood flow, which can help with healing.
  1. Protect your skin from getting injured. Calcium deposits can develop if you injure your skin. These can be painful. They feel like firm bumps underneath the skin.
    If you get calcium deposits, it’s important to protect the skin that has calcium deposits from getting bumped or injured. This could cause a calcium deposit to break through the skin, which could be very painful and cause an infection.
    Tattoos also injure your skin, so dermatologists recommend that people who have scleroderma not get a tattoo. This includes saying no to permanent makeup.
  1. Gently clean affected skin. Skin with scleroderma is dry and fragile. When washing, you want to be gentle. Use a mild fragrance-free cleanser, applying it gently with your fingertips. Rinse the cleanser away with warm water.
  1. Moisturize dry skin. Scleroderma can make the skin very dry. Dry skin is more easily injured. Infection and sores can develop.

    To reduce dry skin, dermatologists recommend that you:

If your child has morphea on the head or neck, regular eye exams from an ophthalmologist (eye doctor) are necessary to watch for possible damage. Aggressive treatment can stop irreversible eye damage.

  1. Use a humidifier when the air feels dry. This can reduce dry skin.
  1. If you smoke, stop. Smoking wreaks havoc on the skin. It also worsens other symptoms of scleroderma like Raynaud phenomenon (extreme sensitivity to cold and stress). Because two types of scleroderma — limited cutaneous scleroderma and diffuse cutaneous scleroderma — can cause scarring in the lungs, stop smoking is especially important.
  1. Avoid beauty treatments that tighten your skin or increase the amount of collagen in your skin. The skin is hard and tight because your body is producing too much collagen. For this reason, you want to avoid collagen injections, a type of filler used to plump up the skin. You also want to avoid skin care products that contain collagen.

Images
Image 1: Thinkstock
Image 2: J Am Acad Dermatol. 2012;67(4):769-84.

Reference
Mayes MD and Ho KT. “Understanding and managing scleroderma.” Scleroderma Foundation. Last accessed February 2015.

© 2019American Academy of Dermatology. All rights reserved. Reproduction or republication strictly prohibited without prior written permission. Use of these materials is subject to the legal notice and terms of use located at https://www.aad.org/about/legal

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