Localised Scleroderma
This only affects the skin, causing one or more patches of hardened skin to develop on the body.
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The treatment for morphoea depends on the type (linear, plaque or generalised), the person’s age and whether it has spread deeper underneath the skin.
What treatment can you expect?
The aim of treatment is to stop progression, shorten the duration of disease and avoid tissue damage. Several courses of treatment may be needed over a lifetime, especially when localised scleroderma begins in childhood. There is no cure for localised scleroderma, but in most cases, it is not a serious condition. It is generally thought to get better by itself, but this may take many years. There are several treatment options available, that will vary depending on the type, extent and depth of involvement, the activity of your condition and your wish (or not) to be treated.
Before any treatment decisions are made, you will be assessed by your dermatologist and/or a rheumatologist. They will ask you questions about your medical history and whether you have been a smoker, as well as carry out a physical examination and a range of tests. Your doctor will then discuss a treatment plan with you, and there may be several options available.
Types of treatments
Different treatments are used for different types of localised scleroderma.
- Strong steroid creams or ointments are often used
and may help to reduce inflammation and speed up the resolution of the plaques. They are applied once or twice a day and can be used safely for between four and 12 weeks, depending on the steroid strength. - Vitamin D analogues, which are usually used twice-aday for several months. Combinations of vitamin D analogues and steroids are available as ointments or foams and can also be effective.
- Immunosuppressive ointments including tacrolimus and pimecrolimus used twice daily.
- Imiquimod cream are also sometimes recommended.
If you have superficial but disseminated plaque morphoea, especially if the plaques are inflamed or waxy and thickened, light treatment may be recommended.
Light treatment may also be recommended in some cases of superficial linear morphoea or as an additional treatment in pansclerotic morphoea.
- Ultraviolet A1 (UVA1) is the most effective form of light therapy as it penetrates deep into the skin and can lead to improvement. However, it is not widely available in the UK.
- PUVA light treatment, where you soak in a bath of psoralen, have psoralen gel painted on to the affected areas, or take a psoralen tablet prior to exposure to UVA light, is a much more widely available form of light therapy, which can also give good results.
In most cases of active linear morphoea and pansclerotic morphoea, especially if there is involvement of muscles (myositis), or fascia (fasciitis), tablet treatments and/or steroid infusions are recommended.
These treatments can also be offered if you have disseminated plaque morphoea if cream or light treatments have not been helpful.
- Infusions of steroids (methylprednisolone) are sometimes given at the start, or monthly for the first threeto-six months.
- Steroid tablets may also be given over the first few months and may be needed for longer (e.g., 18 months), if you have fasciitis.
The main tablet treatments used are immunosuppressants.
- Methotrexate is used “first line” as there is the most evidence for its effectiveness in morphoea (in adults and children).
- Mycophenolate mofetil is used if methotrexate is contraindicated or not tolerated. In some cases, ciclosporin can be added and more recently hydroxychloroquine, an anti-inflammatory, has been found to have some beneficial effects.
- Physiotherapy to maintain mobility.
- Manual lymphatic drainage massage may be beneficial.
- Stem cell fat transfer and fillers may be helpful to reduce asymmetry, especially in head and neck disease
- Orthopaedic and plastic surgeons may also need to be involved in the treatment of joint contractures and limb asymmetry.
What is the outlook (prognosis)?
Plaque morphoea
With this 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; however, a brown stain may remain and sometimes a small depressed area of skin. In some cases the plaques remain for many years.
Linear localised scleroderma
This tends to persist for longer but may improve after several years. It may come and go and may flare up after a long time of being settled. However, linear localised scleroderma, especially the en coup de sabre subtype, may become more extensive with time. This may cause severe contractures (hardening, shortening and tightening of the skin, muscle and/or connective tissue) 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.
