Juvenile Localized Scleroderma

What is juvenile localized scleroderma?

Juvenile localized scleroderma is an autoimmune condition that causes a child’s skin to become unusually thick and hard. Another name for localized scleroderma is morphea.

The word scleroderma comes from two Greek words: “sclera,” meaning hard, and “derma,” meaning skin. The term “localized” means that it mainly involves the skin, connective tissue, muscle and bone.

Scleroderma causes your child’s immune system to attack their skin. As a reaction, your child’s skin inflames (swells). The inflammation can trigger connective tissue cells to produce too much collagen, a protein that’s a major part of many tissues. Excess collagen can lead to fibrosis, which is like scarring. You’ll notice fibrosis forming on your child as a discolored patch of skin.

What are the types of localized scleroderma?

There are different types of localized scleroderma, each with its own symptoms:

• Circumscribed or plaque morphea: It affects the skin and occasionally the tissue just under the skin. The patches (plaques) of affected skin are small, few in number and appear on only one or two areas of the body.
• Linear morphea: Long patches appear in lines across the body or travel in the same direction as your child’s arms and legs. The thickened skin can affect underlying bone and muscle and limit your child’s ability to move their joints or muscles. This can affect their growth and movement.
• Generalized morphea: There are four or more patches of skin affecting two or more areas of your child’s body (usually the trunk and legs). The skin patches may spread and connect to one another.
• Deep morphea: This is the most harmful type and it’s very rare. Deep morphea occurs in the tissue just under the skin and includes the muscle and bone. This type can cause pain.

How common is juvenile localized scleroderma?

Juvenile localized scleroderma is a rare condition that affects an estimated 1 in 100,000 children in the U.S.

What are the symptoms of juvenile localized scleroderma?

Symptoms of juvenile localized scleroderma form from a buildup of collagen in your child’s body. This visibly targets your child’s skin, affecting the color and texture of a small area of skin (similar to a bruise). Symptoms progress or change over time and could include:

1. Patches (also known as plaques) that form on your child’s skin that appear red-to-purple with a normal skin texture and thickness.
2. The patches become hard and swollen (bigger), with a white or yellow waxy (shiny) center, surrounded by a pink or purple halo.
3. In late stages, the affected areas of skin become dark or light (brown to white).

Severe types of localized scleroderma cause deep patches within your child’s tissues and can cause symptoms like:

• Pain and discomfort.
• Limited range of motion of their limbs and muscles.
• Dental or vision problems.

Patches that form on your child’s skin can decrease over time but usually need treatment. Patches can return (flare up) after they’ve gone away.

Can juvenile localized scleroderma become systemic?

Systemic means that the condition affects the entire body. Juvenile localized scleroderma isn’t systemic. Symptoms of this condition don’t affect your child’s entire body. Instead, the condition is “localized,” which means it only targets certain parts of the body.

What causes juvenile localized scleroderma?

The exact cause of scleroderma is unknown. There are likely environmental factors such as exposures, infections or trauma that may contribute, in addition to an overactive immune system.

What causes my child’s skin to harden?

Children diagnosed with localized scleroderma have hard patches of skin on their bodies because they have too much collagen. Collagen is a protein that structurally supports your tissues and gives them elasticity, which allows them to stretch and bend. If your child has too much collagen in their tissues, their skin thickens, tightens and loses its flexibility.

Who is at risk of juvenile localized scleroderma?

Localized scleroderma can affect anyone at any age, but the term “juvenile” means that the condition affects children. Children who are white and children assigned female at birth (AFAB) are slightly more likely to have the condition.

How is juvenile localized scleroderma diagnosed?

Your child’s provider will diagnose localized scleroderma after a physical examination and learning more about your child’s symptoms.

Their provider might order tests to confirm the diagnosis, which could include:

• Skin biopsy to understand more about the affected area of skin.
• Magnetic resonance imaging (MRI) can be used to measure the depth of lesions and look for other features suggesting associated conditions.

How is juvenile localized scleroderma treated?

The goal of juvenile localized scleroderma treatment is to stop inflammation, keep the condition from getting worse and reduce the formation of hard (fibrous) tissue. Treatment is unique to each child and depends on how severe their diagnosis is.

Treatment includes some combination of the following options:

• Corticosteroids: Topical cream, oral or intravenous (IV) medications.
• Topical creams: Calcipotriene, tacrolimus, pimecrolimus or imiquimod.
• Immunomodulating agents: Methotrexate, mycophenolate mofetil or abatacept.
• Skin creams containing lanolin: Help relieve dryness and itching.
• Phototherapy: Uses ultraviolet rays to help soften plaques. Your child’s provider will caution against the use of this treatment since it can prematurely age the skin or lead to skin cancer.
• Physical therapy and massage: Improves muscle strength and maintains joint mobility.
• Plastic surgery: Repairs cosmetic effects of the condition once the disease is fully in remission.

Fibrous tissue is the end stage of inflammation. Once the inflammation stops, your child’s body is capable of reabsorbing some of the fibrous tissue and the skin can soften again.

Your child will have the most positive outlook if their condition receives treatment early, when they first get their diagnosis.

How do I help my child manage their symptoms?

You can help your child protect their skin by:

• Avoiding soaps and detergents with strong scents that may irritate the skin.
• Using gentle, sensitive skin moisturizers and lotions.
• Using warm water when taking a bath or shower.
• Running a humidifier in your home.
• Wearing sunscreen when outdoors.

How can I prevent juvenile localized scleroderma?

There’s no known way to prevent juvenile localized scleroderma since the cause is unknown.

What can I expect if my child has juvenile localized scleroderma?

Juvenile localized scleroderma often affects skin tissues and doesn’t harm major organs. At some point, your child’s hard skin may soften and only hyperpigmented (darkened) areas may have symptoms. Old patches of skin affected by scleroderma can recur (come back), or new skin patches can develop.

Some children may end up with hardened skin, while others may experience damage to their skin or limbs. Your child might need surgery if the condition didn’t respond well to treatment. Your provider won’t recommend surgery until your child finishes growing and the condition is inactive.

Does localized scleroderma affect my child’s lifestyle?

Children diagnosed with localized scleroderma can lead a normal life. They should attend school, play sports and take part in extracurricular and family activities. In general, there are no specific limitations to physical activities children want to do (as long as it’s safe). Exercise doesn’t harm children with this condition and it can help build strength in their muscles.

Your child doesn’t need to eat or drink special foods and beverages to treat their condition. Instead, they should eat healthy, well-balanced meals.

Does juvenile localized scleroderma affect my child’s life expectancy?

Children diagnosed with localized scleroderma have an excellent prognosis (outlook). The condition doesn’t affect your child’s life expectancy.

When should I see my child’s healthcare provider?

Visit your child’s healthcare provider if you notice patches on your child’s skin increasing in size, causing pain, or affecting their lifestyle by limiting their range of motion or their ability to move their muscles.

What questions should I ask my doctor?

• Are there side effects to the treatment you recommend?
• What types of lotions or creams do you recommend for my child’s skin?

What is en coup de sabre morphea?

This is a type of linear morphea, which is a type of localized scleroderma. If linear morphea symptoms affect your child’s face or scalp, the condition is known as “en coup de sabre” (French for “cut by a sword”). Symptoms of en coup de sabre appear as an indented, vertical, colorless line of skin on their forehead.

A note from Cleveland Clinic

You may feel worried about your child’s juvenile localized scleroderma diagnosis, but it won’t affect their ability to have a fulfilling childhood. Their symptoms might cause social challenges if they’re uncomfortable with the way symptoms affect their skin. As their caregiver, offer support for your child as they grow. Remind them they’re unique, rather than looking at their diagnosis as a negative part of their life. If your child has trouble moving, playing or participating in normal activities, talk to your child’s healthcare provider.