Scleroderma

What is scleroderma?

From: MedlinePlus, National Library of Medicine

Scleroderma means "hard skin." It's the name of an autoimmune disease that causes inflammation and thickening in the skin and other areas of the body. This inflammation causes you to have areas of tight, hard skin. Scleroderma may affect just one area of your body, or it can affect many systems in your body.

What are the types of scleroderma?

From: MedlinePlus, National Library of Medicine

There are two main types of scleroderma:

• Localized scleroderma only affects your skin and the muscles and tissues just under your skin.
• Systemic scleroderma, which is also called systemic sclerosis, is a more serious type. It affects many parts of your body and can damage your blood vessels and internal organs, such as your heart, lungs, and kidneys.

What causes scleroderma?

From: MedlinePlus, National Library of Medicine

The exact cause of scleroderma is unknown. Researchers think that several factors may play a part in causing the disease:

• Your genetics. Certain genes can increase the chance that you will develop scleroderma. They may also play a role in which the type of scleroderma you have. Scleroderma is not passed from parent to child, but you are more likely to develop it if a close relative has it.
• Your environment. Exposure to certain things in the environment, such as viruses or chemicals, may trigger scleroderma.
• Immune system changes. When your immune system changes, it can trigger your cells to make too much collagen in the body. Too much collagen causes patches of tight, hard skin.
• Hormones. Hormonal or immune system differences between women and men might play a part in the disease.

Who is more likely to develop scleroderma?

From: MedlinePlus, National Library of Medicine

Anyone can get scleroderma, but certain factors may make you more likely to develop it:

• Your sex. Scleroderma is more common in women than in men.
• Your age. The disease usually appears between the ages of 30 and 50.
• Your race. Scleroderma can affect people of all races and ethnic groups, but the disease can affect African Americans more severely.

What are the symptoms of scleroderma?

From: MedlinePlus, National Library of Medicine

The symptoms of scleroderma are different for each person, depending on the type of scleroderma you have:

• Localized scleroderma usually causes patches of thick, hard skin in one of two patterns: Patches in firm, oval shapes that stay in one area or spread to other areas of skin. This is called morphea. Lines of thickened or different colored skin that run down your arm, leg, and, rarely, on the forehead. This is called linear scleroderma.
• Systemic scleroderma can cause problems with your internal organs as well as your skin. It can cause symptoms such as: Thick, tight skin on your fingers Fatigue Raynaud's phenomenon, a narrowing of blood vessels in the hands or feet Damage to your internal organs, including your digestive system, lungs, kidneys, and heart

How is scleroderma diagnosed?

From: MedlinePlus, National Library of Medicine

There is no single test for scleroderma. The symptoms can vary from person to person and can be similar to those of other diseases. This can make scleroderma hard to diagnose.

To find out if you have scleroderma, your health care provider:

• Will ask about your symptoms and medical history
• Will do a physical exam
• May order blood tests, including an ANA (antinuclear antibody) test
• May do a skin biopsy
• May do other tests, such as imaging tests, to check for organ damage

What are the treatments for scleroderma?

From: MedlinePlus, National Library of Medicine

There is no cure for scleroderma, but treatments can help control your symptoms and limit damage. The treatments may include:

You may need to see specialists to help treat your disease. Many people with scleroderma will see a rheumatologist. This is a doctor who specializes in rheumatic diseases such as arthritis and other inflammatory or autoimmune disorders. Dermatologists, who specialize in conditions of the skin, hair, and nails, may also play an important role in treating the disease. And if you have organ damage, you may need to see other specialists.

You can also help manage some of your symptoms, for example by:

• Medicines to help decrease swelling, manage pain, control other symptoms, and prevent complications.
• Physical or occupational therapy to help with pain, improve muscle strength, and teach you ways to help with daily living.
• Regular dental care, because scleroderma can make your mouth dry and damage connective tissues in your mouth. These problems can speed up tooth decay and cause your teeth to become loose.
• Dressing warm and avoiding cold or wet environments
• Quitting smoking (if you smoke)
• Putting on sunscreen before you go outdoors
• Using moisturizers on your skin to help lessen stiffness
• Avoiding hot baths and showers, harsh soaps, and household cleaners
• Getting regular physical activity

Which doctor should you see?

The suggested department for discussing Scleroderma is Rheumatology, with a rheumatologist as the relevant type of clinician. General physician / Family Medicine; paediatrician for children. Referral depends on symptoms.

This is an editorial referral starting point. The appropriate clinic depends on the person’s age, symptoms, previous diagnosis and local services. The first clinician can decide whether another specialty or a team is needed; a department label does not confirm the diagnosis.

How to prepare for an assessment

Bring a short timeline of the main symptoms: when they first appeared, whether they are constant or episodic, what seems to change them, and how they affect daily activities. Include previous reports, discharge summaries, current medicines and supplements, allergies, and any relevant family history. A dated record is more useful than trying to match every feature in an online article.

Ask the clinician what is already established and what remains uncertain. If a test is suggested, ask what question it answers, what its limitations are and how the result would change the next step. The information here is not an instruction to arrange every possible test. In children, bring growth, developmental and school information if it is relevant to the concern.

• Are the findings inflammatory, structural or due to another mechanism?
• Is there evidence that other organs need assessment?
• How will function and any treatment-related risks be monitored?

Treatment discussions and follow-up

Where the source describes treatments, these are an overview of possible care, not a prescription for an individual. Ask which option applies to the confirmed diagnosis, what benefit is expected, what adverse effects to watch for and how progress will be assessed. Availability, approvals and local practice can differ from the country described in the source.

Before leaving the appointment, clarify the next review date, who will communicate results, and whom to contact if the situation changes. Discuss difficulties with sleep, work, school, mobility, eating or emotional wellbeing when these are relevant. Practical support may require coordination between the treating clinician and other services.

When to seek emergency help

Severe breathing difficulty, collapse, new stroke-like symptoms, a seizure that is prolonged or repeated without recovery, uncontrolled major bleeding, or an immediate risk of self-harm require emergency help. In India, call 112 or reach the nearest emergency department. This is a general, non-exhaustive warning list; it is not a condition-specific triage tool.