Scleroderma

Codes

ICD-10

M34

ICD-11

4A42.Z Systemic sclerosis, unspecified

Description

What is scleroderma?

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?

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?

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 raise your chance of getting scleroderma. They may also affect which type of scleroderma you have. Scleroderma is not passed from parent to child, but you are more likely to get it if a close relative has it.
• Your environment. Things around you may trigger scleroderma, such as certain chemicals, viruses, or medicines. However, a trigger cannot be found for most people.
• Immune system changes. When your immune system attacks healthy tissue by mistake, it can trigger your cells to make too much collagen, which 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?

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 is more common and can be more severe in African Americans.

What are the symptoms of scleroderma?

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 has two kinds. Limited cutaneous scleroderma affects less of your skin while diffuse cutaneous scleroderma spreads further and is more likely to damage your organs.

Symptoms can include:

• Thick, tight skin on your fingers
• Fatigue
• Raynaud's phenomenon, a narrowing of blood vessels that makes your fingers or toes turn white, blue, or red and feel numb or painful from cold or stress
• Joint pain and stiffness
• Trouble swallowing, heartburn, or shortness of breath
• Damage to your internal organs, including your digestive system, kidneys, and heart

Most people with systemic scleroderma have some loss of lung function.

How is scleroderma diagnosed?

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?

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

• Medicines to help lower swelling, manage pain, control other symptoms, and prevent complications.
• Physical or occupational therapy to help with pain, improve muscle strength, and learn easier ways to do everyday tasks.
• 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.

You may need to see specialists to help treat the disease. Many people with scleroderma will see a rheumatologist. This is a doctor who specializes in autoimmune and other diseases of the bones, joints, and muscles. Dermatologists, a doctor who specializes in skin diseases, may also play an important role in treating the disease. You may need to see other specialists if scleroderma affects your organs. It can also help to talk with a mental health professional about living with a long-term illness.

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

• 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
• Seeing your dentist for regular checkups

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Source: MedlinePlus, National Library of Medicine.

Related terms in the cited sources

Medicines with an official indication for this condition

• Bosentan Treatment
• Nintedanib Treatment

In alphabetical order.

Next to it in ICD-10

• M30Polyarteritis nodosa
• M32Systemic lupus erythematosus (lupus)
• M33Dermatomyositis and polymyositis
• M41Scoliosis
• M45Axial spondyloarthritis, unspecified
• M46Other inflammatory spine diseases

Neighbouring codes in the classification, not a medical link.

External resources

• MedlinePlus — Scleroderma
• MedlinePlus — Esclerodermia (es)
• WHO — ICD-10 M34
• WHO — ICD-11 4A42.Z