Localized Scleroderma (Juvenile): Types & Symptoms, Treatment

Overview

Scleroderma, literally meaning “hard skin,” describes a group of conditions that causes the skin to tighten and harden. Juvenile localized scleroderma (JLS) is one type of juvenile scleroderma. It is more common and less severe than the other and also the general form called systemic scleroderma. Localized scleroderma is also known as morphea.

JLS can cause damage to the skin, muscle, bones and joints, but less to internal organs. Girls are slightly more likely to have the disease than boys. It can affect any ethnic groups, but Caucasians are at a higher risk. The best estimate for the number of patients with this disease is 50 children out of every 100,000.


Cause

JLS is an autoimmune disease, that is to say, the immune system mistakes the body’s own tissues as foreign invaders and attacks them, causing inflammation in the skin. It can trigger connective tissue cells to produce too much collagen. Excess collagen can lead to fibrosis, or a hardening and scarring of the tissues.

Risk factors for JLS include genes, gender and environment. Certain genetic mutation can make the individual more susceptible to the disorder although it is not a hereditary disease. Females are at a greater risk of getting affected than males. Environmental factors, such as trauma, infections, drug or chemical exposure, may play a role in triggering the disease.


Types & Symptoms

Signs and symptoms of JLS depend on the type of it. The types of JLS and their related symptoms are:

  • Circumscribed or plaque morphea.

This is the least harmful type of JLS. It mainly affects the skin, and occasionally the tissue just under the skin. The plaques are small, few in number, and appear on only one or two areas of the body.

  • Linear scleroderma.

This is the most common type of JLS. Long plaques may appear in lines across the body or travel in the same direction as the arms and legs.

  • Generalized morphea.

In this condition, there are four or more plaques affecting two or more areas of the body (usually the trunk and legs).

  • Bullous morphea.

When the skin blisters or bubbles, we call it bullous. This condition can occur because of some type of trauma at the site of the plaque, or because the normal flow of lymphatic fluid is blocked.

  • Deep morphea

This is the most harmful form but is very rare. It usually occurs in the tissue just under the skin, including muscle and bone.


Complications

The prognosis for JLS is much better than that for systemic scleroderma, because JLS would not affect internal organs, which can be life-threatening in some cases. However, some other conditions and problems may occur if JLS is left untreated. They include:

  • Disfigurement
  • Eye inflammation, eyelid or dental problems
  • Headaches
  • Seizures
  • Brain problems
  • Limited joint movement
  • Muscle atrophy
  • Reflux of stomach contents
  • Deformed, shorter, or smaller limb or portion of their face or scalp for children with linear scleroderma

To prevent these complications, timely diagnosis and proper treatment are rather important.


Diagnosis

Doctors usually diagnose JLS based on the patient’s medical history and results of physical examination. Further tests and imaging techniques that can help confirm the diagnosis include:

  • Skin biopsy at the affected part of the body
  • Infrared thermography to measure temperature differences between normal skin and the lesion, in order to determine abnormal areas of tissue growth
  • Ultrasound to measure blood flow through the tissues and depth of lesions
  • Magnetic resonance imaging (MRI) to measure the depth of lesions

Specific tests used should depend on the symptoms and severity of the condition.


Treatment

There is no known cure for JLS. The disease can go into remission, meaning the abatement of symptoms, but the timing of different types of JLS varies. Some lesions that do not extend into deeper tissues may go into remission within a few years, whereas linear scleroderma lesions—especially on the head—can remain active for many years.

The goal of treatment is to control the inflammation and keep the disease from getting worse. Treatment usually lasts 2-3 years and includes some combination of the following options:

  • Corticosteroids to reduce inflammation
  • Immunosuppressive drugs such as methotrexate and mycophenolate mofetil for patients with linear scleroderma, lesions on the head or deep lesions
  • Phototherapy using ultraviolet (UVV/UVB) rays to help soften plaques
  • Physical and occupational therapy to improve muscle strength and maintain joint mobility

Surgery may be needed for patients with severe pain or limitation and can improve the appearance of patients with severe facial lesions.


Keywords: juvenile localized scleroderma (JLS); morphea.

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Can Scleroderma Lead to Lung Disease?

Can Scleroderma Cause Death?

What is the Prognosis of Scleroderma?

What is the Best Diet for Scleroderma?

Can You Show Me Some Pictures of Scleroderma?

* The Content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.