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Systemic Sclerosis

Systemic Sclerosis

What is scleroderma? — Scleroderma means "hard skin." It is the medical term for a group of conditions that affect the skin and tissues that support the organs inside the body, called "connective tissue."
There are 2 main types of scleroderma. "Localized" scleroderma affects the skin and tissue just under the skin. "Systemic" scleroderma can affect the skin and organs inside the body, including the heart, lungs, kidneys, and the digestive system. The digestive system includes all the structures and organs that handle and process food, such as the stomach and intestines.
What are the symptoms of systemic scleroderma? — The most common symptoms of systemic scleroderma include:
A condition called "Raynaud phenomenon," which causes the fingers and toes to turn white or purple-blue in response to cold or stress
Puffy skin that slowly becomes hard and thicker than normal
Joints that become stiff, because the skin around them isn't able to stretch as well as it used to
Small white lumps in or under the skin on the fingers – These are called "skin deposits." They contain calcium and might also be called "calcinosis."
Less common symptoms that can happen when other organs are affected include:
Acid reflux – This is when the acid that is normally in your stomach backs up into your esophagus, the tube that carries food from your mouth to your stomach.
Trouble swallowing
Shortness of breath
Will I need tests? — Maybe. Your doctor or nurse might be able to tell if you have it by learning about your symptoms and doing an exam. But you might need tests, including:
Blood tests
Skin biopsy – For this test, a doctor takes a small sample of your skin. Another doctor then looks at the skin cells under a microscope to check for signs of scleroderma.
A chest X-ray
Breathing tests, also called "pulmonary function tests" – These tests measure how well the lungs are working.
How is systemic scleroderma treated? — Treatments for systemic sclerosis include:
Tests every few weeks or months to check your blood pressure and check how well your kidneys and lungs are working
Prescription medicines to treat symptoms in different parts of the body
Surgery to remove calcinosis
Is there anything I can do on my own to feel better? — Yes. You can use special creams with "lanolin" (a greasy substance found in wool) to help keep your skin moist. If itching is a problem, you can take over-the-counter antihistamines, such as diphenhydramine (sample brand name: Benadryl).
What if I want to get pregnant? — If you have scleroderma, you might have a harder time getting pregnant than women without the disease. You also might be more likely to have a miscarriage. (Miscarriage is when a pregnancy ends before a woman has been pregnant for 20 weeks.)
During pregnancy, some of your symptoms (such as Raynaud phenomenon) might get better. But other symptoms (such as heartburn) might get worse. If you have kidney problems caused by scleroderma, pregnancy can be very dangerous and even life threatening.
What will my life be like? — Because scleroderma affects the way you look and, in some cases, your ability to do everyday things, the disease can cause stress and worry. Your doctor can refer you to a counselor or a local scleroderma support group for advice on coping with the condition.
All topics are updated as new evidence becomes available and our peer review process is complete.
This topic retrieved from UpToDate on: Mar 30, 2020.
Topic 83677 Version 6.0
Release: 28.2.2 - C28.105
© 2020 UpToDate, Inc. and/or its affiliates. All rights reserved.

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