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Morphoea

What are the aims of this leaflet?
This leaflet has been written to help you understand more about morphoea. 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 do not damage internal organs.  However they may last for many years before they soften and fade.

What causes morphoea?
The cause of morphoea is not known, but its hardness is due to too great a production of collagen, a protein found in the fibres that give normal skin its strength. Rarely morphoea seems to have been 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, in whites, and in 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?
In morphoea, slightly raised plaques of thickened skin develop slowly and expand outwards. Their surface is usually smooth, and their centres are whitish or ivory coloured.  The edges of new areas often have a violaceous or lilac colour. Older areas tend to become brownish.

Plaques of morphoea range from 2 to 15cm in diameter. They may be single but usually there are several of them: rarely large numbers cover much of the skin (generalised morphoea).  The plaques may lose their hairs and sweat glands so the skin may feel dry.  Usually the plaques are round or oval but sometimes they are long and narrow (linear morphoea).  If they involve a whole arm or leg, structures deep to them (fat, muscle and even bone) may be at risk of growing poorly and developing contractures that limit the movement of joints, but this is very uncommon. In one variety of morphoea, a narrow groove runs up over the forehead into the scalp, where it shows up as a line of hair loss. 

How will 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, there is no cure for morphoea. However, in most people it is not serious. Morphoea may progress for several years (usually 3 – 5) but then tends to “burn out”, getting better by itself, and eventually in some cases the skin returns to normal.

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. They are applied thinly once a day and can be used safely for many weeks. They may be used under an occlusive dressing or can also be injected into the margins of plaques.

In the 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). Light therapy such as PUVA (using a psoralens medicine followed by ultraviolet A light) may also be used to treat extensive morphoea. UVA1 is a specialised form of ultraviolet therapy: it is not widely available and its value is still being assessed.  If any contractures develop, surgery and long-term physiotherapy is helpful.  Many other treatments have been used for morphoea, but there is little evidence that they are effective.

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:

Email: kim@sclerodermasociety.co.uk

Web links to other Internet sites:

www.emedicine.com/med/topic3132.htm

www.dermnetnz.org/immune/morphoea.html

(Whilst every effort has been made to ensure that the information given in this leaflet is accurate, not every treatment will be suitable or effective for every person. Your own doctor will be able to advise in greater detail.)

PATIENT INFORMATION LEAFLET
PRODUCED MARCH 2005