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Morphoea

MORPHOEA (LOCALISED SCLERODERMA)

What are the aims of this leaflet?

This leaflet has been written to help you understand more about morphoea (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 is a rare skin disorder/ disease / complaint where areas of skin become much thicker and firmer than normal. It is sometimes called ‘localised scleroderma.’  This term originates from two words ‘sklero’ meaning ‘hard’ and ‘derma’ meaning skin.  It should not be confused with systemic sclerosis which is a different disease that affects internal organs and the blood circulation as well as the skin. Morphoea usually affects a few small areas of the skin. Very rarely, it can occur over larger areas of the body in a form called ‘generalised morphoea.’  

What causes morphoea?

The cause of morphoea is unknown, but it is not contagious. It is not hereditary but seems to be more common in people with autoimmune complaints and these can run in families. The affected areas of skin become hard because of over-production of collagen fibres. These give the skin its normal strength and structure, so when excess collagen is made, the skin feels harder and more rigid than usual. Morphoea usually starts without any apparent trigger, but it has been noted to happen after a skin injury such as an insect bite, a burn or viral infections.   

It may start at any age including childhood and in adult-hood, it is more common in women than men. Morphoea usually affects white skinned people and is rare in people of African-American ancestry.

What does morphoea look like?

Morphoea usually appears as bruise-like pink patches of skin that thicken and turn pale and waxy in the middle, leaving a pink/ pale purple border. The skin feels firmer than normal when gently pinched. Sometimes the affected areas of skin turn slightly darker and beige coloured. Hair is usually lost from the affected areas and the sweat glands stop working so the skin feels dry. In rare cases, fatty tissue and muscle under the morphoea disappears so the skin becomes indented and stuck onto the underlying bone.     

The affected areas usually range from 2 to 15 cm in diameter and are oval in shape (like a thumb print or palm print). This is called plaque morphoea. In children, morphoea usually appears as a thickened strip of skin called ‘linear morphoea’. This usually runs along part of the torso or a limb. It can also affect the forehead to the scalp and cause a grove in the skin with hair loss (sometimes referred to as ‘en coup de sabre’).              

What are the symptoms?

Morphoea usually develops slowly and there may be few symptoms. The affected area(s) can be itchy, uncomfortable or numb. When morphoea affects the skin on a limb or over a joint, it can restrict growth and mobility and in severe cases can cause long-term disability. 

How is morphoea diagnosed?

A dermatologist can usually diagnose morphoea by examining the skin because it has a distinctive appearance. A skin biopsy (a small piece of the skin, removed under a local anaesthetic) can be taken from the affected area if there is uncertainty or to rule out other skin complaints. Blood tests may also be helpful.   

Is there a cure for morphoea?

There is no known cure for morphoea and it typically lasts for several years so is considered to be a chronic skin disorder. 

How can morphoea be treated?

The treatment for morphoea depends on the type (linear, plaque or generalised), the patients age and whether it has spread underneath the skin.   

Plaque morphoea does not always need treatment because it usually improves on its own after a few years. However, strong steroid creams or ointments or a non-steroid cream such as Tacrolimus are sometimes used as they may relieve any irritation and stop the patches enlarging. Intralesional injections of steroid (into the affected skin) can also help.

Early treatment is important for linear and generalised morphoea to prevent later problems especially loss of mobility. Options include ultraviolet therapy / ‘PUVA’ oral or intravenous steroids and Methotrexate. In addition to care from a dermatologist, expert treatment may be needed from a plastic surgeon or orthopaedic surgeon, rheumatologist and physiotherapist.

Self care (what can I do?)

Using a moisturiser on the affected areas can help relieve dryness of the skin and irritation.

Where can I find out more about morphoea?

Web links to other internet sites:

www.dermnetnz.org/immune/morphoea.html

Links to patient support groups:

Scleroderma & Raynaud’s Association
18-20 Bride Lane,
London,
EC4Y 8EE
Tel: 020 7000 1925
Email: info@sruk.co.uk
Web: https://www.sruk.co.uk/

For details of source materials used please contact the Clinical Standards Unit (clinicalstandards@bad.org.uk).

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: individual patient circumstances may differ, which might alter both the advice and course of therapy given to you by your doctor.

This leaflet has been assessed for readability by the British Association of Dermatologists’ Patient Information Lay Review Panel

BRITISH ASSOCIATION OF DERMATOLOGISTS
PATIENT INFORMATION LEAFLET
PRODUCED MARCH 2005
UPDATED MAY 2010, JANUARY 2014, 
JANUARY 2017
REVIEW DATE JANUARY 2020

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