Scleroderma Symptoms: Recognizing Scleroderma

scleroderma symptoms

Image: unggoonk / Shutterstock

Scleroderma is a chronic autoimmune and rheumatic disease. It’s one of those conditions that is incredibly varied from person to person, with scleroderma symptoms literally ranging from mildly annoying to life-threatening. It’s also pretty rare with estimates hovering around 300,000-500,000 scleroderma patients in the United States. One thing should be obvious from that statistic alone: it’s not contagious. It’s not cancerous either. And like many similar conditions in the autoimmune and rheumatic families, they aren’t really sure of what causes scleroderma either.

We can learn a little about it just from the name alone. It comes from Greek: “sclero” means hard and “derma,” of course, means skin. But what exactly does “hard skin” mean? And how can that possibly become life-threatening?

Scleroderma Symptoms

Hardened skin is actually one of the most visible manifestations, according to the Scleroderma Foundation. More specifically, it’s hardening connective tissue which is comprised of many kinds of proteins, including collagen. You see, as an autoimmune disease, the immune system turns against itself. In the case of scleroderma, the cells make excess collagen as if they are treating an injury. But there is no injury.  Usually, one of the first places it can be noticed is on the fingers and hands. Indeed, the abnormal build-up of tissue in the skin can cause the skin to tighten so much, that the fingers curl and the patient loses the hand mobility.

While not every patient has the same experience, many experience a change in their fingers. Cold or stress causes their fingers to change colors due to a sensitivity. Also, their fingers and hands will become stiff and puffy. Johns Hopkins Medicine, which has an entire center solely focused on scleroderma, explains: “Finger color changes are caused by spasm and narrowing of blood vessels. This occurs because of excess collagen that has narrowed the blood vessels and over reaction of the skin blood vessel to cold temperatures and emotional stress. The cold sensitivity and color changes are called Raynaud’s (pronounced RAY-KNOWDS) phenomenon. Raynaud’s phenomenon is a common condition. Most people with Raynaud’s phenomenon will NOT develop scleroderma.”

Recall that scleroderma symptoms can even be life-threatening in some situations. Johns Hopkins adds that the reason for that has to do with the volume of skin involved. In other words, the more skin affected by scleroderma, the more serious the condition becomes.

Who Gets Scleroderma?

Even though scleroderma isn’t genetic, other family members tend to have other autoimmune and rheumatic diseases. Interestingly, Johns Hopkins adds, “African Americans and Native Americans generally have more severe scleroderma than Caucasians. These findings suggest a hereditary (genetic) component to scleroderma and other autoimmune disorders.” So, at the very least, there certainly appears to be some sort of predisposition that runs in certain families.

The American Autoimmune Related Diseases Association says that, “of the 50 million Americans living and coping with autoimmune disease (AD), more than 75 percent of them are women.” Scleroderma is no exception, effecting a disproportionate number of women with estimates at six out of every seven patients. It develops most frequently between ages 22-55, but infants and the eldery have also been known to get it. And this is a really good time to emphasize the wide variety associated with scleroderma. These stats are merely the most common, but the rest of the data ranges all over the place. In fact, the Scleroderma Foundation says, “There are many exceptions to the rules in scleroderma, perhaps more so than in other diseases.” With that in mind, you will probably need to see both a neurologist and a dermatologist to be certain of a diagnosis.

How to Treat Scleroderma

Remember, there is no cure. That means your options for dealing with this condition fall into the category of management. An added difficulty to the fact that no two scleroderma stories are alike is that there are multiple kinds of this disease, each of which require a different sort of treatment. So, the first thing to do is identify which kind you have, what stage it’s in, and what organs are being effected. For example, one type can cause inflammation of the joints, muscles, or the lining of the heart or lungs. Thus, it typically responds well to antiinflammatories and corticosteroids. While another type of scleroderma inflammation relates to the tissue caused by the damage of the disease itself. Antiinflammatories and corticosteroids can actually cause more harm in these phases of scleroderma so they have to be used very precisely.

Johns Hopkins recommends other treatments such as immunosuppresive therapy, drug therapy of vascular disease (widespread with scleroderma), and anti-fibrotic agents.

Further Reading

For a more in-depth understanding of the comprehensive care related to scleroderma, Johns Hopkins Scleroderma Center offers a download of Chapter 23 of Systemic Sclerosis (pdf) by Dr. Laura Hummers and Dr. Fred Wigley.

Comments

comments