Scleroderma Located, Morfea in Children 4.33/5 (6)

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Linear scleroderma

Localized scleroderma in children 4-9 years old who developed Parry Romerg's syndrome. Lee YJ, 2015. CC BY NC 3.0

Definition

Sclerosis means hardness and derma means skin. There are several types of scleroderma among both children and adults.

  • Localized scleroderma (non-systemic) is different from Systemic sclerosis which to a greater extent injures internal organs
  • Localized scleroderma occurs in various forms and is also called Morfea
  • The disease is limited to the skin and tissue under the skin

Localized scleroderma in children

Among children, this localized form (non-systemic scleroderma) occurs approximately 10 times more often than that Systemic form (systemic sclerosis).

  • The disease attacks the skin either patchy (plaque or generalized morphea) or as a hard, tight band down the skin (linear morphea / linear scleroderma, see below)
  • A form of blisters (bullless morphea) also occurs
  • The symptoms are initially red and slightly warm skin as a sign of rheumatic inflammation of the skin
  • Eventually, the affected area becomes harder and brownish or whitish. more here (Wikipedia)

Linear scleroderma

Linear scleroderma in an arm. Sartori-Valinotti JC, Autoimmune Dis (2013). CC BY 3.0

Linear scleroderma is the most common form of localized scleroderma. The most common location is on one leg, but the disease can also be on one arm or on the face (Coup de sabre).

  • The skin change appears as a stroke or may eventually resemble a scar. At 90% only one side of the body is attacked. The disease process can also include the tissue under the skin, including the muscles and the skeleton. This can cause growth disruption in one leg, arm or face, scalp and skull with subsequent asymmetry. Internal organs are not attacked.
  • Over a few years, many people experience that the skin areas again become softer. However, relapse in childhood occurs.

Linear scleroderma in the forehead. Sartorin-Valinotti JC, Autoimmune Dis (2013). CC BY 3.0

Blood tests

Blood tests for localized scleroderma are often normal, but some with active disease may have increased the number of eosinophilic white blood cells. Also ANA (antinuclear antibody) may be elevated (in 20-40%), but Anticentromer / CENP or Scl-70 (as by Systemic sclerosis) is uncommon (2-3%).

Differential diagnosis

Treatment

The treatment varies and must be considered by a specialist. By just a little patchy changes (morpha), medications are not necessary. Smaller skin changes can be camouflaged with makeup. Some children receive cortisone cream (for example Dermovate) on the skin.

  • Linear scleroderma is more often treated with systemic cortisone (Prednisone) tablets and Methotrexate (tablets or injections). Experience indicates that the treatment reduces growth disorders in some patients
  • Physical therapy may be appropriate to prevent tight skin causing permanent stiffness of the joints, and one may strengthen any weak muscles
  • If the growth disorder causes a difference in leg length (more than 1,5 - 2cm), the orthopedist can consider surgical treatment (Epiphysiodesis) is relevant
  • A European specialist group (SHARE) here made recommendations. They emphasize the following
    • For active disease with severe prognosis, Methotrexate is recommended
    • In this case, you should also consider combining Methotrexate with Prednisolone
    • Mycophenolate is an option if Methotrexate cannot be used
    • Light treatment with medium-dose UVA1 can be attempted to soften the skin
    • Reference: Zulian F, 2019

Medical prognosis

For localized scleroderma, normal life expectancy is expected.

  • The disease often subsides during 3-5 years, but relapse and longer disease course are seen by linear scleroderma

Linear scleroderma in adults

  • Linear scleroderma can also begin among adults, but less frequently than in children (reference: Marizano AV, 2003)

Literature



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