scleroderma

Scleroderma means hard skin. It is a group of diseases that cause abnormal growth of connective tissue. Connective tissue is the material inside your body that gives your tissues their shape and helps keep them strong. In scleroderma, the tissue gets hard or thick. It can cause swelling or pain in your muscles and joints.

Symptoms of scleroderma include :

• Calcium deposits in connective tissues
• Raynaud's phenomenon, a narrowing of blood vessels in the hands or feet
• Swelling of the esophagus, the tube between your throat and stomach
• Thick, tight skin on your fingers
• Red spots on your hands and face

No one knows what causes scleroderma. It is more common in women. It can be mild or severe. Doctors diagnose scleroderma using your medical history, a physical exam, lab tests, and a skin biopsy. There is no cure, but various treatments can control symptoms and complications.

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

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