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Patient Information Leaflet

Morphoea and Localised Scleroderma

What are the aims of this leaflet?

This leaflet has been written to help you understand more about morphoea and localised scleroderma. It tells you what it is, what causes it, what can be done about it, and where you can find out more about it.

What is morphoea?

Morphoea, also known as ‘localised scleroderma’, is uncommon. The word ‘scleroderma’ means ‘hard skin’, and this is the main feature of morphoea. Areas of morphoea are thicker and firmer than the rest of the skin. Morphoea is ‘localised’ in the sense that its plaques appear only in the skin and there is no damage to internal organs. However, the skin signs may last for many years before they soften and improve.

What causes morphoea?

The cause of morphoea is not known. The hardness of skin is due to excessive production of collagen, a protein in skin that gives normal skin its strength. Rarely morphoea may be triggered by an event such as a minor injury, a tick bite, or a viral infection such as chickenpox, but usually it comes up for no apparent reason. It can affect anyone but is most common in children, Caucasians and females. Morphoea is not contagious.

Is morphoea hereditary?

No.

What are the symptoms of morphoea?

Usually there are none, so morphoea may not be noticed for a while. Sometimes it is itchy.

What does morphoea look like?

Raised areas of thickened skin (termed ‘plaques’) develop slowly and expand outwards. The skin surface is usually smooth with white or ivory-coloured centres. The edges of new areas often have a violet or lilac colour. Older areas tend to become brown.

The lesions range from 2 to 15 cm in diameter. They may be single but usually there are several of them. Rarely there may be widespread thickening of the skin (termed ‘generalised morphoea’). Hair and sweat glands may be lost over the affected areas and the skin may feel dry. The shape is typically round or oval but may be distributed in a line (termed ‘linear morphoea’). Rarely involvement of structures deep to the skin (fat, muscle and bone) may be affected. In one variety of morphoea, a narrow groove runs up over the forehead into the scalp (termed ‘en coup the sabre’), where it shows up as a line of hair loss.

How can morphoea be diagnosed?

Your doctor will recognise morphoea by its appearance, but a skin biopsy (a small piece of the skin, removed under a local anaesthetic) will usually be taken and looked at under the microscope in the laboratory to confirm the diagnosis. Blood tests may be needed in more severe cases.

Can morphoea be cured?

No. However, in most people it is not serious. Morphoea may progress for several years (usually 3–5 years) but then tends to “burn out” and spontaneously improves.

How can morphoea be treated?

Plaque morphoea, the commonest form, does not always need treatment, as it will usually improve by itself. However, strong steroid creams or ointments are sometimes used as they may help to reduce inflammation and prevent progression. Steroids may be used under an occlusive dressing or can also be injected into the margins of plaques.

In rare severe cases, oral or intravenous steroids may be given as well as other therapies such as hydroxychloroquine, ciclosporin or methotrexate (covered in other leaflets in this series). Ultraviolet light therapy such as PUVA (using a psoralen medicine followed by ultraviolet A light) may also be used to treat extensive morphoea. UVA-1 is a specialised form of ultraviolet therapy. It is not widely available and its value is still being assessed. If limb movement is affected surgery and physiotherapy may be helpful. Many other treatments have been used for morphoea, but there is little evidence on their efficacy.

What can I do?

General skin care, with the regular use of emollients and soap substitutes, may help. You may also wish to talk about your condition with other people who have it too (see below).

Where can I find out more about morphoea?

Link to patient support group:

Scleroderma Society www.sclerodermasociety.co.uk
Email: kim@sclerodermasociety.co.uk

Web links to other Internet sites:

www.emedicine.com/med/topic3132.htm
www.dermnetnz.org/immune/morphoea.html

The British Skin Foundation fund vital research into all skin diseases.  To find out how you can help, please visit the British Skin Foundation website here.

This leaflet aims to provide accurate information about the subject and is a consensus of the views held by representatives of the British Association of Dermatologists: its contents, however, may occasionally differ from the advice given to you by your doctor.

BRITISH ASSOCIATION OF DERMATOLOGISTS
PATIENT INFORMATION LEAFLET
PRODUCED MARCH 2005
UPDATED MAY 2010