Advertisement

Last Updated: September 11, 2017

Limited Scleroderma (CREST Syndrome)

Overview

Limited scleroderma (also known as CREST syndrome) is a type of scleroderma, which as the name implies is characterized by thick, hardened skin. In the more severe form, this connective tissue disorder can affect the function of internal organs (e.g., esophagus, heart, lungs, gastrointestinal tract, muscle and joints) and even become life threatening. Limited scleroderma is milder form of the disease and affects roughly 50% of scleroderma patients. It is often referred to as CREST syndrome, with each letter representing one of five common features (see Symptoms) of the illness:

calcinosis in a patient with CREST syndrome

The skin hardening associated with limited scleroderma is typically confined to the fingers, hands, lower arms, legs and face. Unlike its more severe form, progression is more gradual and complications involving internal organs are less common. Limited scleroderma can affect the digestive tract, causing difficulty swallowing and frequent heartburn. It can also affect the blood vessels, heart and lungs, leading to hypertension and shortness of breath. There is no cure for scleroderma; treatment is aimed at managing the symptoms.

Causes and Risk Factors

The exact cause of limited scleroderma is not known. Scleroderma is believed to be an autoimmune disease, where the body launches an attack against its own organs. In this case, the immune response seems to prompt the body to overproduce collagen, which is deposited in the skin and other organs, altering their function. The risk factors for limited scleroderma are:

Symptoms

The symptoms of limited scleroderma develop gradually over several years and include:

Diagnosis and Treatment

Limited scleroderma is hard to diagnose due to a wide range of symptoms that overlap with other diseases. Doctors typically review the patient’s medical and family history, and examine the affected skin and organs. Skin biopsy and blood tests may be performed to assist in the diagnosis. Additional tests may be required to assess the state of internal organs, like the heart and lungs.

There is no cure for limited scleroderma. The following treatments are used to manage the symptoms and avoid complications:

Prevention

There is no way to prevent limited scleroderma. Those with a high risk of developing the disease can practice vigilance for the known symptoms.

Sources

Additional Resources

Home Health Testing Guides

Testing Company Reviews

Related Topics

Authored by: Tina Shahian, PhD