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What Is Segmental Vitiligo?

Posted on May 10, 2022

Segmental vitiligo (SV) is a unique type of vitiligo, an autoimmune skin disorder that causes skin depigmentation (loss of color). Skin depigmentation happens when a person experiences a loss of melanocytes. Melanocytes cells produce melanin, the pigment responsible for skin color. When a person loses melanocytes, white patches form in their skin and hair.

Vitiligo affects 0.5 percent to 1 percent of the worldwide population. The U.K’s National Health Service reports segmental vitiligo accounts for up to 30 percent of all vitiligo seen in children.

How Is Segmental Vitiligo Different From Other Forms of Vitiligo?

Segmental vitiligo, also called unilateral vitiligo, has several features that make it distinct from other types of vitiligo. Notably, SV is often limited to one side of the body, while other types of vitiligo usually cause depigmentation on both sides of the body.

Most types of vitiligo slowly advance across the skin over time. However, SV advances rapidly (usually over six months to a year) and then abruptly stops progressing. In about half of SV cases, the condition involves more than just the skin. Someone with SV can also experience depigmentation that affects their eyebrows, eyelashes, and nose hairs, as well as the rest of the hair on their face and body.

The subtype SV differs from other types of vitiligo because it is not commonly associated with other autoimmune diseases. The subtype SV also does not usually occur alongside other skin diseases. However, SV can coexist with other types of vitiligo. When that happens, it’s called mixed vitiligo.

Symptoms of Segmental Vitiligo

The primary symptom of segmental vitiligo — like other types of vitiligo — is the appearance of patches of completely white skin. Some 50 percent of people with SV also see depigmentation in the hair on their bodies — including their eyebrows or eyelashes. These depigmented patches occur exclusively on either the left or right side of the body. Patches of SV typically appear on one primary area, such as on a person’s head, neck, torso, arm, or leg.

In someone with SV, one small area of depigmentation grows rapidly over weeks or months before it stops. In most cases, SV patches stop growing within a year. But the patches may also progress for longer. Conversely, they may only progress for a few days.

Patches of vitiligo can sometimes be itchy and may have redness at their borders.

What Causes Segmental Vitiligo?

The cause of segmental vitiligo remains unknown, but researchers and doctors believe several mechanisms might play a role in its development. For one, autoimmunity appears to contribute to SV by causing the body’s immune system to attack and destroy pigment-producing cells — melanocytes.

Based on the specific pattern of SV patches, the condition may be tied to unusual cell changes that possibly occurred as an embryo develops. The nervous system may also play a role in the formation of SV. Nerve cells in the skin may release chemicals that contribute to the loss of melanocytes.

The onset of vitiligo can be triggered by stress, skin damage, or certain chemical exposures. Rare cases of drug-induced segmental vitiligo have been reported. In particular, the drug infliximab (branded as Remicade, Renflexis, and more) has been associated with case reports of segmental vitiligo.

Diagnosis of Segmental Vitiligo

To diagnose segmental vitiligo, your doctor records your medical history, performs a physical exam, and runs tests to rule out other possible causes of skin depigmentation.

Several things in your medical history can help your doctor make a diagnosis. For example, a family history of vitiligo, a personal history of autoimmune disease, and recent stress, illness, or skin injury (including rashes or sunburns) may contribute to someone’s SV symptoms.

The pattern of SV depigmentation is distinct. Through a physical exam, your doctor will use the location and extent of your patches to help determine your particular type of vitiligo.

Your doctor may also use ultraviolet light (specifically, a Wood’s lamp) to help examine your skin and rule out certain other diseases or conditions. For example, infections such as tinea versicolor, albinism, damage from chemical exposure, and other skin conditions (such as pityriasis alba) can appear similar to vitiligo when studied in normal light. Ultraviolet (UV) light also helps to see when depigmented areas develop on people with light skin.

Your doctor may also collect a skin biopsy and order blood tests to help diagnose vitiligo and specific subtypes.

Treatment of Segmental Vitiligo

You typically don’t need to treat vitiligo unless your patches bother you. In that case, different options may help reduce the appearance of your vitiligo.

Segmental vitiligo tends to be more difficult to treat than generalized vitiligo. Still, research has shown that several vitiligo treatments can be effective. Medication, ultraviolet light therapy, skin grafts, or a combination of therapies can have excellent results in restoring skin pigmentation in SV.

Medication

Medical treatment for SV includes topical and systemic corticosteroids (steroids) and topical tacrolimus (Protopic), an immunosuppressive drug. These treatments can restore pigmentation in some people with SV.

Other medications are being considered, too. Some medications being tested to treat vitiligo include the class of immunosuppressive drugs called Janus kinase (JAK) inhibitors.

Light Therapy

Light therapy, including laser phototherapy and narrowband UVB phototherapy, can also promote skin repigmentation. Phototherapy may be combined with corticosteroids, tacrolimus, or other drugs.

Surgery

Surgery is another treatment option for people with segmental vitiligo — including children. That’s due, in part, to the nature of SV. It doesn’t spread indefinitely. Given that its destruction of melanocytes is limited, SV usually responds well to transplantation. In fact, one doctor from the Vitiligo Clinic & Research Center at UMass Chan Medical School noted they saw success with transplants 80 percent to 95 percent of the time.

In some cases, transplanting hair follicles from an area of unaffected skin can restore melanocytes to depigmented areas. Select skin cells critical for making pigment — melanocytes and keratinocytes — can also be collected from a person’s own skin and transplanted to their patches of SV.

Mental Health Care

Vitiligo of any type can be difficult to live with. Segmental vitiligo may not respond to treatment, and living with visible patches of colorless skin can affect a person’s emotional health. Addressing the anxiety and depression that can occur as a result of having vitiligo is an important part of treatment and can improve a person’s quality of life.

Talk With Others Who Understand

MyVitiligoTeam is the social network for people with vitiligo and their loved ones. On MyVitiligoTeam, nearly 10,000 members come together to ask questions, give advice, and share their stories with others who understand life with vitiligo.

Are you living with segmental vitiligo? Share your experience in the comments below, or start a conversation by posting on MyVitiligoTeam.

In partnership with the Global Vitiligo Foundation, which strives to improve the quality of life for individuals with vitiligo through education, research, clinical care, and community support.

Posted on May 10, 2022

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October 25, 2023
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Victor Huang, M.D., Global Vitiligo Foundation is the director of phototherapy at UC Davis Health. Learn more about him here.
Kristopher Bunting, M.D. studied chemistry and life sciences at the U.S. Military Academy, West Point, and received his doctor of medicine degree from Tulane University. Learn more about him here.

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