Localised Scleroderma Explained

Localised scleroderma causes one or more patches of hard skin. There are different types. The common plaque type usually causes no problems (apart from sometimes an unsightly appearance) and tends to fade in time. The linear type can cause problems to underlying tissues such as muscles and bones.

Often no treatment is needed for the plaque type but treatment may be advised in more severe cases and for the linear type.

What are the symptoms of localised scleroderma?

Localised scleroderma usually comes on gradually. Areas of the skin may be thickened and discoloured, and hair may be lost over the area of scleroderma. There are different types of localised scleroderma, and the most common ones are:

Plaque morphoea – these are oval patches that can range from 2 to 20 cm across. They start off mauve in colour, then gradually become white. Older patches may become brown.

The surface is smooth, shiny and hairless. One to three plaques may develop in different areas of the skin. This type mainly affects adults and usually has no other symptoms or problems, although the affected skin can look unsightly.

  • Superficial morphoea – is similar to the plaque type. It is typically seen as symmetrical mauve-coloured patches, usually in skin folds of the groin, armpits or under breasts. type is most commonly found in middle-aged women.
  • Linear scleroderma – usually occurs on an arm or leg of a child. It is a long, narrow area of thickened skin. In more severe cases the tissues just beneath the skin are affected which may cause scarring (contractures) underneath the skin.
  • En coup de sabre – this is a deep form of linear scleroderma affecting the scalp and temple. The name comes from its shape, meaning ‘like a sword cut’. Hair is lost over the affected skin, and the skull bone may be shrunk underneath it.
  • Generalised morphoea – this is a rare type where there are more plaques spread around different areas of the body.

Are there any complications of localised scleroderma?

Usually, localised scleroderma does not cause any complications. It is not linked to the systemic sclerosis form of scleroderma, and does not involve internal organs.

Some types of localised scleroderma may affect not only the skin, but the tissues directly underneath it such as bone or muscle.

This only happens in the deeper types of scleroderma (linear scleroderma and en coup de sabre). In this situation, the scleroderma can affect growth in the underlying tissues, and so may cause some degree of deformity.

About one third of people with deep localised scleroderma have this type of problem.

Rarely, if the affected skin is located on the head, the brain or eye can be affected.

Who gets Localised Scleroderma?

Localized scleroderma can occur at any age and in any race, but is more common in Caucasians. Most patients who develop scleroderma are female.

Environmental factors, such as trauma, infections, or drug or chemical exposure, may play a role, but not for most patients. The disease is not contagious.

The disease is not passed on directly from parent to child by any one gene, though certain genes may make a child more likely to develop localized scleroderma.

Localized scleroderma is a rare disease, and the exact number of patients with this disease is not known. The best estimate is that 50 children out of every 100,000 will develop localized scleroderma.

How is Localised Scleroderma diagnosed?

Scleroderma usually is diagnosed by a rheumatologist or dermatologist based on the patient’s history and physical examination.

There are no specific laboratory studies to diagnose localized scleroderma, but tests often are done to evaluate level of inflammation and problems related to localized scleroderma, and to make sure the patient does not have another condition.

A skin biopsy may be done to confirm the diagnosis.

How is Localised Scleroderma treated?

Treatment varies depending on the patient’s disease activity, lesion location and extent, and whether there are related problems. Careful clinical evaluation is the primary method for monitoring scleroderma.

X-rays and computerized tomography (CT) scans are used to look at bone abnormalities. Thermography can detect differences in skin temperature between the lesion and normal tissue.

Ultrasound and magnetic resonance imaging (MRI) can aid soft tissue assessment.

Current treatment is focused on controlling inflammation, as this decreases the risk of serious problems such as differences in limb length, areas of sunken skin on the face, limited joint movement, and an internal organ problem.

Patients with linear scleroderma, lesions on the head, deep lesions, or widespread disease, are usually treated with systemic medications that suppress the immune system.

These medicines include methotrexate, which is given by injection or taken by mouth once a week, and corticosteroids, which is taken by mouth (prednisone) or given by infusion (intravenous methylprednisolone).

A recent randomized clinical trial showed that methotrexate was better than placebo at maintaining disease control after initial corticosteroid treatment.

Other immunosuppressive medicines include mycophenolate mofetil, cyclosporine and tacrolimus. More work is needed to determine the best therapy for localized scleroderma.

Immunosuppressive medicines can increase a patient’s risk for developing an infection and have other possible side effects; these will be reviewed by your doctor.

For patients with mild superficial disease, topical medications often are used to control the inflammation and soften the skin.

These medications include corticosteroids, calcipotriene, tacrolimus, pimecrolimus and imiquimod. Moisturizers may help protect and soften the skin.

Phototherapy has been used to treat patients with widespread, superficial disease. Both UVB and UVA have been reported to help.

More study is needed to evaluate the potential side-effects from exposing children to large amounts of ultraviolet light.

Physical and occupational therapy to improve strength and function is important for patients with muscle weakness, limb length differences, and limited joint movement.

Surgery is not recommended during treatment of active disease. Surgery may be needed for patients with severe pain or limitation, and can improve the appearance of patients with severe facial lesions.

However, because the skin is abnormal there can be poor wound healing, and surgery may trigger a flare of disease in some cases. Cosmetic makeup can be used to make the lesions less noticeable.

There is no known cure for localized scleroderma. The disease can stop on its own (remission), but the timing of this varies.

Circumscribed morphea lesions that do not extend into deeper tissues may go into remission within a few years, while linear scleroderma lesions—especially on the head or scalp—can remain active for many years or even throughout childhood.

It is important that patients who have lesions involving deeper tissues continue to be monitored at least yearly, even after treatment is stopped, because the disease can come back (relapse).

Broader Health Impacts: Assoiated Problems

The overall prognosis is much better for localized scleroderma than for systemic sclerosis, because life-threatening internal organ involvement is extremely rare.

However, localized disease can cause disfigurement, and the skin hardening can cause discomfort and sores as well as problems such as limited joint movement.

In addition, many children can have problems of other organs or tissues. Those with linear scleroderma are at a higher risk for severe growth problems, such as developing a deformed, shorter or smaller limb or portion of their face or scalp.

Children with linear scleroderma lesions on their face or head can develop eye inflammation, eyelid or dental problems, headaches, seizures and other brain problems.

They need regular eye examinations and—in some cases—MRI evaluation of the brain and/or eyes.

Other problems include arthritis, limited joint movement, muscle atrophy, and reflux of stomach contents. Children with pansclerotic morphea can develop chronic skin ulcers and may be at risk for squamous cell carcinoma.

Living with Illness; Lifestyle Changes

Most children with localized scleroderma don’t need to make major lifestyle changes. All affected children should go to school.

Some accommodations may be needed for children with severe disease affecting their ability to walk or write easily.

Encourage affected children to remain active, but some activities—such as contact sports—should be limited in patients at risk for skin breakdown or in those with extremely limited movement in major joints.

What is the outlook (prognosis)?

With the plaque morphoea type of localised scleroderma, in many cases the plaques last 3-5 years before softening and fading.

The affected areas of skin may gradually go back to normal, but a brown stain may remain and sometimes a small depressed area of skin. However, in some cases the plaques remain for many years.

Linear-type localised scleroderma tends to persist for longer, but may improve after several years. It may come and go, and flare up after a long time of being settled.

However, linear-type localised scleroderma, especially the en coup de sabre subtype, may become more extensive with time.

This may cause severe contractures that result in limited movement and permanent disability of an arm or leg. Damage to underlying brain and eye tissue is a potential, serious complication of the en coup de sabre subtype.

Source & More Info: patient.co.uk and rheumatology.org

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