sclerodermas

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.

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.

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.

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.

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

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

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

• 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.

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:

• 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

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Your connective tissue supports many different parts of your body, such as your skin, eyes, and heart. It is like a "cellular glue" that gives your body parts their shape and helps keep them strong. It also helps some of your tissues do their work. It is made of many kinds of proteins. Cartilage and fat are types of connective tissue.

Over 200 disorders that impact connective tissue. There are different types:

• Genetic disorders, such as Ehlers-Danlos syndrome, Marfan syndrome, and osteogenesis imperfecta
• Autoimmune disorders, such as lupus and scleroderma
• Cancers, like some types of soft tissue sarcoma

Each disorder has its own symptoms and needs different treatment.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Raynaud phenomenon is a condition that affects your blood vessels (the tubes that your blood flows through). It causes your blood vessels to narrow, which decreases blood flow. This is called a Raynaud episode or "attack." The attacks usually affect your fingers and toes, causing them to become cold and numb. They may also change color, usually to white or blue. These attacks happen in response to cold temperatures or stress.

Raynaud phenomenon may also be called Raynaud disease or Raynaud syndrome.

There are two types of Raynaud phenomenon:

• Primary Raynaud phenomenon is the more common type. Its cause is unknown.
• Secondary Raynaud phenomenon is usually caused by another disease or problem, such as lupus or scleroderma. Other causes may be exposure to cold or certain chemicals. The type can be more serious than the primary type.

Researchers don't know exactly why some people develop Raynaud phenomenon. But they do understand how the attacks happen. When you are exposed to cold, your body tries to slow the loss of heat and maintain its temperature. To do this, the blood vessels in the top layer of your skin constrict (narrow). This moves blood from those vessels near the surface to vessels deeper in the body. But when you have Raynaud phenomenon, the blood vessels in your hands and feet react to cold or stress by narrowing quickly. They also stay narrowed for a long time.

Anyone can develop Raynaud phenomenon, but some people are more likely to develop it:

• Primary Raynaud phenomenon has been linked to:
  • Your sex. Women get it more often than men.
  • Your age. It usually develops in people younger than age 30. It often starts in the teenage years.
  • A family history of Raynaud phenomenon. You are more likely to develop Raynaud phenomenon if you have a family member who has it.
• Secondary Raynaud phenomenon has been linked to:
  • Certain diseases. These include lupus, scleroderma, rheumatoid arthritis (RA), carpal tunnel syndrome, and connective tissue disorders.
  • Certain medicines. Medicines that treat high blood pressure, migraines, and attention deficit hyperactivity disorder (ADHD) may cause similar symptoms to Raynaud phenomenon or make your symptoms worse.
  • Work-related exposures, such as repeated use of vibrating machinery (such as a jackhammer), or exposure to cold or certain chemicals.

Raynaud attacks most often happen when you get cold, for example when you grab something cold from the freezer or go into an air-conditioned building on a warm day. Attacks usually affect your fingers and toes. But sometimes they can affect other parts of your body, such as your ears, nose, lips, or nipples.

An attack causes the skin to become cold and numb. Your skin may also turn white or blue due to a lack of oxygen. As the blood flow returns, your skin may tingle, throb, or turn red. An attack may last a few minutes or a few hours. If you have darker skin, you may not be able to easily see the skin color changes.

For many people, especially those with the primary type, the symptoms are mild. People with the secondary type often have more severe symptoms. They may develop skin ulcers (open sores caused by poor blood flow) or skin infections.

There is no specific test to diagnose Raynaud phenomenon. To find out if you have it, your health care provider:

• Will take your medical history and ask about your symptoms.
• Will do a physical exam.
• May order blood and other lab tests to check for other conditions which could be causing your symptoms and/or to help decide which type of Raynaud phenomenon you have.

Most people with Raynaud phenomenon can keep their symptoms under control by avoiding getting cold. But if this is not enough, medicines and, in some cases, surgical procedures can help.

Secondary Raynaud phenomenon is more likely to be serious and to need more treatments. It's also important to treat the condition or problem that is causing your Raynaud phenomenon.

You may need to see a specialist such as a rheumatologist, a doctor who treats diseases of the joints, muscles, and bones.

Raynaud phenomenon cannot be prevented, but you can help prevent attacks and manage your symptoms by:

• Placing your hands or feet in a warm place when you have an attack. This could mean putting them under warm (not hot) water or under a heating pad.
• Keeping your body, especially your hands and feet, warm in cold weather.
• Avoiding triggers, such as certain medicines and stress.
• Quitting smoking (or not starting smoking).
• Managing stress.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Pulmonary hypertension, sometimes called PH, is a serious condition that affects the blood vessels in the lungs. It develops when the blood pressure in your lungs is higher than normal.

Your heart pumps blood to your lungs so they can add oxygen to the blood. The blood goes back to your heart. From there, it travels to the rest of your body so that your tissues can get the oxygen that they need.

The blood moves from your heart to your lungs through blood vessels called pulmonary arteries. If the pulmonary arteries become damaged, narrowed, or blocked, the blood does not flow through them as well. This can increase the blood pressure in the arteries and cause pulmonary hypertension.

There are different types of pulmonary hypertension, including pulmonary arterial hypertension (PAH). The different types are based on what caused the disease.

Pulmonary hypertension can develop on its own or be caused by another condition. Sometimes the cause is unknown or is not clear.

Some of the possible causes include:

• Heart diseases, including left-sided heart failure and congenital heart disease
• Lung diseases such as COPD (chronic obstructive pulmonary disease), interstitial lung disease, emphysema, and sleep apnea
• Other medical conditions such as:
  • Liver diseases
  • Sickle cell disease
  • Pulmonary embolism (blood clots in the lungs)
  • Connective tissue disorders like scleroderma

Certain factors can make you more likely to develop pulmonary hypertension, such as:

• Your age. The risk increases as you get older. The condition is usually diagnosed between ages 30 and 60.
• Your environment. Being exposed to asbestos or having certain infections caused by parasites can raise your risk.
• Your family history and genetics. Certain genetic disorders, such as Down syndrome, congenital heart disease, and Gaucher disease, can raise your risk of pulmonary hypertension. So can a family history of blood clots.
• Your lifestyle habits.Smoking and illegal drug use can raise your risk of developing pulmonary hypertension.
• Certain medicines. For example, some medicines used to treat cancer and depression can make you more likely to develop pulmonary hypertension.
• Your sex. Pulmonary hypertension is more common in women than in men.

The symptoms of pulmonary hypertension are sometimes hard to recognize and are similar to the symptoms of other medical conditions. So sometimes it can take years for someone to get diagnosed with pulmonary hypertension.

The symptoms of pulmonary hypertension may include:

• Shortness of breath
• Chest pain or pressure
• Dizziness that may lead to fainting
• Fatigue
• Swelling of the abdomen, legs, or feet
• Heart palpitations (the feeling that your heart is pounding or beating too fast)

Pulmonary hypertension can get worse over time and lead to serious problems, including:

• Anemia, which can cause your body to not get enough oxygen-rich blood
• Arrhythmias, problems with the rate or rhythm of your heartbeat
• Blood clots in the pulmonary arteries
• Bleeding in the lungs
• Heart failure
• Liver damage
• Pericardial effusion, a collection of fluid around the heart
• Serious pregnancy complications

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

• Will ask about your medical history and symptoms
• Will do a physical exam, which may include checking your blood oxygen, listening to your heart and lungs, and checking your blood pressure
• Will likely order some tests, such as:
  • Blood tests to look for blood clots, stress on the heart, or anemia
  • Heart imaging tests, such as a cardiac MRI
  • Lung imaging tests, such as chest x-ray
  • An electrocardiogram (ECG or EKG)

Often there is no cure for pulmonary hypertension, but treatments can help manage your symptoms. Your provider will work with you to come up with a treatment plan. It will be based on your needs and the cause of the pulmonary hypertension. The plan may include:

• Healthy lifestyle changes, such as:
  • Healthy eating, which includes eating less salt
  • Regular physical activity, which may be done through pulmonary rehabilitation
• Medicines, such as:
  • Blood thinners
  • Medicines to control the rate blood is pumped throughout the body
  • Medicines to relax blood vessels and allow the blood to flow better
  • Medicine to reduce swelling (diuretics)
• Oxygen therapy
• Procedures to reduce pressure in the heart or pulmonary artery
• In some severe cases, a lung transplant

NIH: National Heart, Lung, and Blood Institute

Your skin is your body's largest organ. It covers the entire outside of your body. There are many ways that your skin protects your body and helps keep you healthy. For example, it:

• Holds body fluids in, which helps prevent you from getting dehydrated
• Keeps out harmful germs, which helps prevent infections
• Helps you feel things like heat, cold, and pain
• Helps control your body temperature
• Makes vitamin D when the sun shines on it
• Shields your body against heat and light

There are many different problems and conditions which can affect your skin. Some of them can cause uncomfortable symptoms, such as itching, burning, redness, and rashes. They might also affect your appearance. Some of the more common skin conditions include:

• Acne, which causes pimples when hair follicles under your skin get clogged up
• Burns
• Cuts and scrapes
• Dandruff, flaking of the skin on your scalp (the top of your head)
• Eczema (atopic dermatitis), which causes inflammation, redness, and irritation of the skin
• Hives, which are red and sometimes itchy bumps on your skin
• Insect bites
• Psoriasis, which causes itchy, scaly red patches
• Skin cancer
• Skin infections

Since your skin protects your body in many ways, it's important to try to keep your skin healthy. For example, you can:

• Wear the right protective equipment, like gloves, long sleeves, knee and elbow pads, or helmets to protect against cuts, bumps and scrapes.
• If you do get a cut or scrape, clean it right away with soap and warm water. Put on a bandage to protect it while it heals.
• When you are spending time outdoors, wear long sleeves and pants and use insect repellant to prevent insect bites.
• Prevent sunburn by covering up and using sunscreen when outdoors.
• Wash your hands often with soap and water.
• When you take a shower or bath, use warm (not hot) water. Use mild cleansers and wash gently (don't scrub).
• Use moisturizers, like lotions, creams, or ointments, to prevent dry skin.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases