Pediatric Scleroderma in Children

What is scleroderma?

Scleroderma means hard (sclero) skin (derma). It is a rare autoimmune disease in which normal tissues are replaced with dense, thick scar tissue. Scleroderma can affect the skin or other organ systems. In children, scleroderma most frequently affects only the skin.

What are the types of pediatric scleroderma?

There are two broad types of scleroderma:

  • Localized scleroderma – most common in children. This form of scleroderma does not change into the generalized forms of scleroderma.
  • Systemic scleroderma (sclerosis) – rare in children

Localized scleroderma

Localized scleroderma is the most common type of scleroderma in children and it usually affects the skin, only occasionally spreading to the underlying muscles.

There are two main types of localized scleroderma: linear scleroderma and morphea.

When the areas of skin affected appear in a band, it is called linear scleroderma. Linear scleroderma most often occurs on an arm or leg and occasionally on the face or neck. It can result in cosmetic problems, growth abnormalities of the affected area and if it crosses joint lines, limitation of joint motion and contractures (inability to straighten the joint).

Skin changes that appear in patches – either as a single patch or in a group – are called morphea. Morphea appears waxy and usually has an ivory or white color.

In both linear scleroderma and morphea, the skin may change to appear white with purple borders.

In addition to skin changes, a child with systemic sclerosis may also develop problems with internal organs (such as the kidneys, heart, lungs and gastrointestinal tract). Children with systemic sclerosis have more widespread skin changes that can result in limited joint movement.

Raynaud’s phenomenon (white, blue and red color changes of the fingers and/or toes upon exposure to cold or stress) is present early in a child with systemic sclerosis, as well as fatigue, joint pain, difficulty swallowing, abdominal pain, heartburn, diarrhea and shortness of breath.

Children with systemic sclerosis should be checked often for high blood pressure, as well as lung, kidney, gastrointestinal and heart problems, to detect and treat potential internal organ involvement.

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