Scleroderma Located, Morfea in Children 4.5/5 (8)

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Definition

Sclerosis means hardness and derma means skin. There are several types of scleroderma among both children and adults. Localized scleroderma (non-systemic) which is described in more detail on this page is different from Systemic sclerosis which to a greater extent damages internal organs. Localized scleroderma occurs in various forms and is also called morphea. The disease is limited to the skin and the tissue beneath the skin.

Localized scleroderma in children

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

Occurrence. Among children, this localized form (non-systemic scleroderma) occurs approx. 10 times more frequent than that Systemic form (systemic sclerosis). Nevertheless, this is a rare condition. In Great Britain, the number of new cases (incidence) is 3,4 per million children annually. Most have the linear type (see below). The disease is somewhat more common among girls than boys with a ratio of 2,4:1 and the average age at the onset of the disease is 7,3 years (reference: Herrick AL, 2010),

Symptoms and investigations

Medical history includes the time of disease onset and spread over time. Raynaud's phenomenon and other symptoms from the hands and feet that are unusual are requested. Symptoms from joints (arthralgia, arthritis) are also not expected.

Clinical. The child's general condition is often unaffected and good. Localized scleroderma attacks the skin either in patches (plaque or as generalized morphea) or as a hard, tight band down the skin (linear morphea / linear scleroderma, see below). A form with blisters (bullous morphea) is the least common. The symptoms initially are an area of ​​red and slightly warm skin. Eventually the affected area becomes harder, brownish or whitish. Raynaud's phenomenon and diffusely swollen fingers are unusual and more reminiscent of Systemic sclerosis.

Blood tests such as CRP, metabolism (f-T4, TSH), liver and kidney function tests are expected to be normal. Individuals with active disease may have an increased number of eosinophilic white blood cells. ANA (antinuclear antibody) can also be elevated (in 20-40%), but Anticentromer / CENP or Scl-70 (as by Systemic sclerosis) is uncommon (2-3%).

Imaging usually show normal findings. In the case of a pronounced attack on the head, an MRI examination can be done to assess how deep the changes go.

Disease Causes

The causes of the disease are unknown, but in the course of the disease it can be demonstrated that cells (fibroblasts) produce too much connective tissue (collagen).

Other diseases at the same time

One sees a slightly increased co-occurrence of classic autoimmune diseases such as inflammation of the thyroid gland (autoimmune thyroiditis), vitiligo with pigment loss and diabetes mellitus type I (reference: Leitenberger IJ, 2009).

Subgroups of localized scleroderma

Localized scleroderma can be divided into five subgroups: Circumscript morphea are limited oval or round indurated, yellow-white areas with a violet halo around them, most often on the body, less often on the arms, legs or face. Generalized morfea consists of four or more changes that are over 3 cm in diameter and distributed in at least three of seven anatomical areas (head-neck, right arm, left arm, right leg, left leg, front or back part of the body). Linear scleroderma  is the most common subgroup among children and young people. A linear, atrophic line is typically seen in the skin over the forehead, skull (cup de sabre), extremity and/or truncus (see more below)Pansclerotic morphea is very rare, but serious because so large parts of the skin are attacked, such as the body, arms and legs, face and scalp. Unlike systemic sclerosis, the hands and feet are spared. Raynaud's phenomenon is also not common. Mixed subtype of morphea has elements of two or more of the above-mentioned subgroups (reference: Zulian F, 2007).

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 among children and adolescents. The most frequent localization is on one leg, but the disease can also be present on an arm or on the face (Coup de sabre).

The skin changes in linear scleroderma may appear as a red, thick line or may eventually resemble a scar. In 90%, only one side of the body is attacked. The disease process can involve the tissue under the skin, including the muscles and the skeleton. This can cause growth disturbance in a leg, an arm or in the face, scalp and skull with subsequent distortion. Internal organs are not attacked. Over a few years, many people experience that the skin areas become softer again. Relapses during childhood occur.

Parry-Romberg syndrome

Parry-Romberg syndrome is a variant of linear scleroderma where the face below the forehead is affected, which easily causes growth disturbance with distortion and problems with jaw function (reference: Arif T, 2020).

Similar conditions / differential diagnoses

Treatment

The treatment varies depending on the severity and must be assessed by a specialist.

Cortisone creams. In the case of only slightly patchy changes (morphea), medication is not always necessary. Minor skin changes can be camouflaged with make-up. Some people get cortisone creams on their skin.

Medication. 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

Physiotherapy may be relevant to prevent tight skin from causing permanent stiffness over joints, and one can strengthen any weak muscles.

SHARE recommendations. A European specialist group (SHARE) has drawn up recommendations. They emphasize the following: In active disease with a serious prognosis, methotrexate is recommended. In that case, you should also consider combining Methotrexate with Prednisolone. Mycophenolate is an alternative if methotrexate cannot be used. Light treatment with medium-dose UVA1 can be tried to make the skin softer. (Reference: Zulian F, 2019).

Medical prognosis

In localized scleroderma, a normal lifespan is expected. The disease often subsides within 3-5 years. An older study showed that 3,8 years after diagnosis, 50% had softer skin (Peterson LS, 1997). Relapses and a longer course of the disease are, however, seen in linear scleroderma. Overall, over 50% with linear scleroderma will have persistently reduced function due to the disease (reference: Piram M, 2013).

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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