Linear Scleroderma

Also known as: localized scleroderma, linear systemic sclerosis.

What is linear scleroderma?

While there are many medical conditions that can present with tightening and thickening of the skin, the term “Scleroderma” (hard skin) is usually used to describe a rare autoimmune disease (where the body’s normal defense mechanisms against bacteria and viruses attacks its own tissues and organs) that causes an increased production of dense, tough, hard, scar-like tissue to replace normal tissue. Two broad forms exist:

  1. Localized, the one most commonly found in children, and which  damages the skin, muscle, bones and joints. There are 3 types of localized scleroderma of which “ linear scleroderma” is one type.

  2. Systemic scleroderma, a more severe form of the disease, rarely seen in children which damages internal organ damage. One form does not turn into the other!

What causes linear scleroderma?

Scleroderma occurs as a result of the overproduction of collagen by the body. Why exactly this occurs, however, is unknown.

What are the signs/symptoms of linear scleroderma?

A shiny, thickened streak of tough darker (or lighter) looking skin that may involve a leg or arm and may spread along a line to feet or hand, and sometimes to head, face, scalp, or forehead. This often affects deeper layers of skin, spreads over joints and may limit the movement of the joint. If extensively covered, it may not allow the limb to grow normally.

What are linear scleroderma care options?

Treatments may include medications such as nonsteroidal anti-inflammatories, corticosteroids, penicillamine and/or immunosuppressive drugs as well as therapies to protect the skin, physiotherapy and exercise, and/or surgery as indicated.

Reviewed by: Ana Margarita Duarte, MD

This page was last updated on: August 19, 2021 03:42 PM

Children's Dermatology

The Division of Dermatology at Nicklaus Children's Hospital focuses on the latest medications and technology available for the specialized treatment of all skin diseases and disorders affecting children of all ages.

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