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Scleroderma: Diagnosis and treatment


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

However, 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:

  • Keep your ability to move a joint (i.e., jaw, finger, or wrist) when thickened skin covers it

  • Minimize tightening of skin over joints

Your dermatologist may refer you for physical therapy

Physical therapy can help you retain the ability to straighten and bend your joints.

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.

Many treatment options

While there are many treatment options, 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:

  • Calcipotriene (may also reduce discolored skin and visible blood vessels)

  • Calcipotriene + a strong corticosteroid

  • Imiquimod

  • Tacrolimus ointment

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.

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

Laser therapy can help get rid of visible blood vessels

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.

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:

  • Methotrexate

  • Corticosteroids that you take by mouth

  • Medicine used to treat malaria

  • Mycophenolate mofetil

  • D-penicillamine

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:

  • Methotrexate

  • A corticosteroid that you take by mouth

  • UVA phototherapy

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.

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.

Side effects are possible with different treatments

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.


Images
Images 1 and 4: Getty Images

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.

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