Scleroderma

For those living with scleroderma and their loved ones, it’s important to understand the illness and what it entails. This short section will describe the most important facts about scleroderma, and why treatment to manage the symptoms may be necessary.

What is Scleroderma?

Scleroderma is an autoimmune disease, which means that the body is attacking itself in ways that are not typical.  There is no cure for it.

In general, scleroderma causes your body to make too much collagen, which can lead to hardening and tightening of the skin and connective tissues (sclero =hard, and derma = skin).  This can cause pain in the hands or other areas, such as in bands around the torso.

Scleroderma can be mild or serious, and can develop at any age.  Symptoms include:

The exact symptoms will depend on whether the disease is localized or systemic, and how severe it is.  Scleroderma may only affect small areas of skin, or it can affect organ systems and be life-threatening.

What Causes Scleroderma?

While anyone can develop scleroderma, it is most common in women who are between 30 and 55 years old.  It can affect people from infancy through the old age.  It’s not necessarily progressive, and may never cause serious issues (depending on the type), or it may be very serious.

There is no known cause of scleroderma.  Physicians don’t know why some develop it and others don’t.  However, because it is most common in middle-aged women, doctors do suspect a hormonal factor is involved.

Scleroderma is not strictly genetic, according to current research, however some subtypes do tend to run in families.

Scleroderma can have many symptoms in common with other autoimmune diseases, so it’s important to be seen at a qualified clinic and receive treatment early to properly manage the symptoms and live a healthy life.

What are the different types of scleroderma?

Scleroderma can be either localized or systemic.  Systemic scleroderma is more serious, but also less common.

Localized Scleroderma

There are two sub-types of localized scleroderma.  These include morphea and linear.

Morphea

In localized morphea scleroderma, patches of hard, tight skin form on the body.  These patches may enlarge or spread over time, but also may spontaneously disappear.

Linear

This is a little more serious, and involves lines of hard, tight skin that form along the body.  It often includes deeper layers of skin and connective tissue.  If it occurs in children, it may affect growth.

Systemic Scleroderma

There are also two subtypes of systemic scleroderma.  These include limited and diffuse.

Limited

This is the less serious of the two, and about 50% of patients have this form.  It is typically slower to develop, and affects fewer of the internal organs.  Most of the symptoms are on the face and hands.  A few patients will go on to develop pulmonary issues (difficulties with breathing and lung function).

Diffuse

This is the most severe form of the disease.  These patients typically have multiple organ systems involved, and the disease may progress faster.

Complications of Scleroderma

The normal path and prognosis of scleroderma varies widely.  Patients with the mildest forms may only need home treatments, such as topical corticosteroids that are prescribed by their doctors.  Others with more severe forms may need a wide variety of both home and clinical treatments in order to manage their symptoms and have better quality of life.

Complications can include:

If you have any concerns about your condition, or the most appropriate treatments, talk to your doctor.  S/he will be able to advise the best course of action to manage your symptoms and promote quality of life.

Sources:

http://www.scleroderma.org/site/PageNavigator/patients_whatis.html#.WisHGUqnGM8

https://www.mayoclinic.org/diseases-conditions/scleroderma/symptoms-causes/syc-20351952

https://www.webmd.com/pain-management/scleroderma#1-2