Scleroderma: Types, Symptoms, Treatment

Overview

Scleroderma, literally meaning “hard skin”, is an autoimmune disease that affects the skin and other parts of the body. An autoimmune disease means that the body’s immune system is unexplainably attacking normal tissues and cells, causing inflammation and abnormalities in these areas. Scleroderma is characterized by skin thickening and tightening, as well as inflammation and scarring of many body parts.

Scleroderma is a relatively rare disorder. In the U.S, about 300,000 people have this disease. Most of the patients are women between the ages of 30 and 50. Although there is no cure for scleroderma, a variety of treatments can help control the symptoms and improve paitent’s quality of life.

Types

There are two types of scleroderma: localized scleroderma and systemic scleroderma. Localized scleroderma is the less severe form of the disease and is more common in patients with scleroderma. Systemic scleroderma, also called generalized scleroderma, is relatively rare but can be life-threatening.

Localized scleroderma mainly affects the skin. Generally, it involves two subtypes: morphea and linear scleroderma. Systemic scleroderma can involve many body parts or systems. There are two kinds of this as well: limited scleroderma and diffuse scleroderma.

Causes

The cause of scleroderma is still unclear. Overproduction and accumulation of collagen in body tissues can trigger the disease. Collagen is a fibrous type of protein that makes up your body’s connective tissues, including your skin. However, doctors don’t know what prompts this collagen accumulation. Experts indicate that the body’s immune system plays a role in this process.

Risk factors for scleroderma include:

  • Sex

Scleroderma affects women more often than men.

  • Age

People aging between 30 and 50 are at higher risk.

  • Ethnic groups

Choctaw Native Americans and African-Americans are more likely than Americans of European descent to develop systemic scleroderma.

  • Gene

Most patients do not have any relatives with scleroderma and their children do not get scleroderma. Research indicates that there is a susceptibility gene. It may raise the likelihood of getting scleroderma, but itself does not cause the disease.

  • Environment

Possibly certain viruses may trigger the development of scleroderma in genetically susceptible people.

Symptoms

The signs and symptoms of scleroderma vary depending on the type and the affected area. Common symptoms include:

  • Muscle weakness
  • Short breath
  • Heartburn
  • Diarrhea
  • Weight Loss
  • Hardened or thickened skin, mostly on the hands and face
  • Cold fingers or toes that may turn red, white, or blue, which is known as Raynaud’s phenomenon
  • Problems in absorbing nutrients
  • High blood pressure from kidney problems
  • Ulcers or sores on fingertips
  • Small red spots on the face and chest
  • Swollen, painful or numb fingers and/or toes
  • Pain and inflammation in the joints

Diagnosis

Scleroderma can be difficult to diagnose because it can take many forms and affect many different parts of the body. Normally, a physical exam comes first. During the exam, your doctor will ask about your medical history and detailed symptoms. Then, he or she may adopt other methods to confirm the diagnosis, including:

  • A blood test to check for certain antibodies associated with the disease
  • X-rays and CT scans to look at bone abnormalities
  • Thermography to detect differences in skin temperature between the lesion and normal tissue
  • Ultrasound and MRI to assess soft issues
  • Biopsy to check for tissue abnormalities

Treatment

There exists no treatment option to cure scleroderma. But with a combination of medications and non-medication therapies, your symptoms can be managed and controlled to lead a normal life.

Medications that can be helpful in treating scleroderma include:

This class of drugs, such as lisinopril, are frequently used to control high blood pressure and to prevent kidney and lung failure.

Some researches indicate that colchicine can help decrease the inflammation and tenderness in the skin.

This type of drugs can help treat heartburn caused by scleroderma.

NSAIDS, like ibuprofen, can help control swelling and pain.

Corticosteroids may help control your immune response to prevent muscle, joint, or internal organ problems.

Physical and occupational therapies can help you manage pain, improve muscle strength and flexibility and maintain independence with daily tasks.

For more severe cases, surgery is the last resort. If finger ulcers caused by severe Raynaud’s disease have developed gangrene, amputation may be necessary. A lung transplant is the option for people who have developed high blood pressure in the arteries to their lungs.


Keyword: scleroderma.

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* The Content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.