Localised Scleroderma

Diseases of the immune system; Diseases of the skin

Localised Scleroderma


The information on the Rare Awareness Rare Education (RARE) Portal is intended for educational purposes only and does not replace professional advice.

Rare diseases typically display a high level of symptom complexity and variability. Individuals diagnosed with the same rare disease may be impacted differently and each person’s experience is unique. Please seek support from qualified healthcare professionals to learn more about the most suitable care and support options for you.

For more information on this disease, please refer to RVA Partner, Scleroderma Australia.1

If you are aware of any additional information that may benefit stakeholders with an interest in this page, or if you notice any broken links or inaccurate information, please let us know via the Contribute page.


Information for this page has been supplied by RVA Partner, Scleroderma Australia.1

Emergency Management

There may be special considerations for the emergency management of individuals living with scleroderma presenting to emergency departments.

The following hospital care plan documents have been published by Scleroderma Australia1, with information produced in collaboration with Scleroderma/Lupus Resource Centre at the Royal Newcastle Hospital, NSW:

Clinical Care Guidelines

Information for referring practitioners can be found here for some states:

Consensus-based recommendations for the management of juvenile localised scleroderma was developed, and published in 2019, by a committee of paediatric rheumatologists from 11 countries, including Australia.



ORPHA:90289 Localised scleroderma

ICD11: EB61.0 Plaque morphoea


Localised scleroderma is a type of Scleroderma, an autoimmune condition that affects connective tissues, which joins and supports other tissues and organs in the body. ‘Scleroderma’ comes from the Greek words ‘skleros’ (hard) and ‘derma’ (skin), as this condition is often characterised by the hardening or thickening of skin.1 This is caused by a reaction of the body’s immune system resulting in too much collagen, an important protein of the connective tissue, being produced. This overproduction of collagen can lead to scarring and damage.1

Whilst other types of scleroderma may affect the skin and internal organs, localised scleroderma is limited to the skin and in some cases, the tissues or muscles beneath the skin.2,3 It rarely affects the internal organs2 and will not progress to the systemic form of scleroderma.3

Localised scleroderma is characterised by the appearance of areas of hard and dry skin on the hands, face, arms or legs, followed by plaques or wide strips depending on the subtype of condition. This can occur on one side of the body, affecting symmetry of the face and limbs.4 Localised scleroderma can also affect the mobility of joints.

The two subtypes of localised scleroderma are:

  • Morphea – presence of round or oval plaques, often with a white appearance and red rim. It is more common in adults and is not always permanent.4
  • Linear scleroderma – presence of wide strips, mainly in the face, chest (thorax), arms and legs. It can also affect the fat and muscles under the skin and lead to muscle wasting (atrophy).4 It is more likely to affect children (juveniles) than adults2,4 and tends to be more severe than the morphea subtype.4

More information about scleroderma can be found at: Scleroderma Australia: Understanding and Managing Scleroderma.


Areas of hard and dry skin may appear on the hands, arms, legs, or face, followed by plaques or wide strips depending on the subtype of condition.4 In the morphea subtype, round or oval plaques, usually white and with a red rim are observed. In linear scleroderma, there are wide strips mainly in the face, chest (thorax), arms and legs. The white strips may present on the face and scalp as a scar-like appearance as if struck by a sword/sabre (en coup de sabre). 2-4 Muscles, fat tissue and skull may also be affected in linear scleroderma.3

Growth of limbs in children and mobility of joints may be affected in localised scleroderma.4  Unlike in the systemic form of scleroderma, there is usually no observation of Raynaud’s phenomenon (narrowing of blood vessels leading to decreased blood flow and symptoms such as numbness in parts of the body) in localised scleroderma.2-4


Scleroderma is an autoimmune condition which causes the body to overproduce collagen. The exact cause of the autoimmune reaction is unknown.


Diagnosis of scleroderma may be made based on clinical examination of symptoms/disease presentation and medical history.

For localised scleroderma, a skin biopsy may be used to detect for accumulation of collagen, thickening of blood vessel walls, and white blood cells around the vessels.4

Antinuclear antibodies that are normally present in individuals with systemic scleroderma are usually not found in people with localised scleroderma.2


There is currently no curative treatment for scleroderma, but there are strategies to assist in the management of symptoms. This may involve different types of medication to suppress the immune system and to treat specific symptoms. Physiotherapy and exercise may help with joint mobility and muscles.2,4

Please speak with your medical team to learn more about management strategies for scleroderma and its associated symptoms. Treatment will depend on an individual’s specific symptoms.

Clinical Care

Healthcare professionals involved in the treatment of localised scleroderma may include general practitioners (GP), rheumatologists, dermatologists, neurologists, wound care nurses, physiotherapists, and psychologists.1,3 The need for different healthcare professionals may change over a person’s lifetime and extend beyond those listed here.

The Australian Scleroderma Interest Group (ASIG) is a collaboration of rheumatologists who operate multidisciplinary scleroderma centres around Australia.


Information about research on scleroderma in Australia can be found at Scleroderma Australia: Australian research into Scleroderma.

The Australian Scleroderma Interest Group (ASIG) is a collaboration of rheumatologists, who in addition to operating multidisciplinary scleroderma centres around Australia, have designed the Australian Scleroderma Cohort Study (ASCS), aimed at creating a national database about people with scleroderma and mixed connective tissue disease (MCTD). They have also established a bio-bank to collect and store biological specimens from patients. More information about the ACSC and the bio-bank can be found at ASIG: ASCS Demographic & Biobank.

Scleroderma Australia: Clinical Trials Understanding Scleroderma provides an in-depth overview of what clinical trials may be like for those living with scleroderma.

Please visit Australian Clinical Trials to learn about clinical trials for scleroderma in Australia; there may not be any clinical trials currently available.

Information regarding clinical trials for scleroderma in other countries can be found at ClinicalTrials.gov; there may not be any clinical trials currently available.

It is best to discuss your interest in any clinical trials with your medical team to determine suitability and eligibility.

Rare Disease Organisation(s)

Scleroderma Australia
Website: https://www.sclerodermaaustralia.com.au/
Phone: 02 9990 5159
Email: [email protected]
Contact form: https://www.sclerodermaaustralia.com.au/contact-us/

Scleroderma Australia is the national representative voice in supporting the scleroderma community across Australia, with its governing committee made up of delegates from different states. Scleroderma Australia also produces a number of informative brochures that explain aspects of scleroderma for medical professionals, people living with scleroderma, their families and friends. These free resources can be found at: Scleroderma Australia: Free brochures and information.

There are different state organisations that provide support to people living with scleroderma:

The Scleroderma Association of New South Wales
Website: https://www.sclerodermansw.org/
Phone: 02 9798 7351
Email: [email protected]

Scleroderma Association of New South Wales provides support for those living in New South Wales, and the Australian Capital Territory.

Scleroderma Victoria
Website: https://sclerodermavictoria.com.au/
Phone: 03 9231 3651
Email: [email protected]

Scleroderma Victoria provides support for those living in Victoria, South Australia, Tasmania, and Western Australia.

Scleroderma Association of Queensland
Website: https://www.scleroderma.org.au/
Phone: 0468 801 021
Email: [email protected]

Scleroderma Queensland provides support for those living in Queensland, and the Northern Territory.

Please note that RVA does not necessarily monitor or endorse each group/organisation’s operational governance.

Social Services

Scleroderma Australia: Understanding Scleroderma and the NDIS provides information to help people with scleroderma apply for funding through the National Disability Insurance Scheme (NDIS) for support and services.

Please visit the Disability Support Information page on the RARE Portal for more information on disability supports, and the National and State Services pages for general information on other social services.

Mental Health

Please visit the ‘Mental Health’ sections listed on the National and State Services pages.


Further information on localised scleroderma can be found at:

  1. Scleroderma Australia. Accessed 16 October 2023. https://www.sclerodermaaustralia.com.au/
  2. Scleroderma Australia. Understanding and Managing Scleroderma 6th ed. 2021. 19p. https://www.sclerodermaaustralia.com.au/wp-content/uploads/2021/07/Understanding-and-Managing-Scleroderma-6th-Edition.pdf
  3. Genetic and Rare Diseases (GARD) Information Center. Localized scleroderma. Accessed 16 October 2023. https://rarediseases.info.nih.gov/diseases/7058/localized-scleroderma
  4. Orphanet. Localized scleroderma. Accessed 16 October 2023. https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=90289
Page Last Updated

30/01/2024 12:36