Doc Talk

Softening a 'hard skin' condition

Early diagnosis and medications are critical to treat the stiff joints and hardening of skin

Ms B was a 33-year-old teacher who had just delivered her first baby and was looking forward to spending time with her newborn daughter.

However, during her confinement, she began to get stiff, painful joints which made gripping the feeding bottle a strain.

Her skin was tight and dry. Her body itched all over.

She lost a lot of weight and increasingly, the skin around her fingers became hard and the area around her elbows became stiff, making her arms inflexible.

Several months later, her fingers turned blue whenever she was in an air-conditioned environment.

I saw Ms B two months after her initial symptoms.

Clinical examination and investigations revealed that she had systemic sclerosis (SSc), more commonly known as scleroderma or "hard skin".

She had extensive hardening of the skin on her face, arms and legs, and painful and swollen joints which limited her movement.

Medications were started immediately to try and control her condition quickly.

It was a difficult time for her, filled with anxiety over how she would cope with her daughter.

With the medications she was taking, she would not be able to continue breast-feeding.

But fortunately, she consulted the doctor very early and received the necessary treatment, so she was likely to have better control of her condition.

SSc is a disorder of the immune system that leads to excessive production of collagen tissue, also referred to as fibrosis. It usually strikes during the prime of life, between the ages of 30 and 50 years.

Between 50 and 200 per million people worldwide are estimated to have the condition and women are four times more likely to develop it than men.

The condition typically affects the skin and blood vessels. The first symptoms are often skin tightness, and patients may notice stiffness and pain in the joints.

While aches and pains in the joints may commonly be thought of as part and parcel of growing old, these "wear and tear" joint pains are usually exacerbated by movements of the joint.

But joint pains relating to autoimmune disorders tend to be associated with swelling, and pain is alleviated with movement and worsens with rest.

Not everyone's condition is as serious as Ms B's, where the skin hardening advanced very rapidly from the hands to the arms, and to the rest of the body.

Some patients barely notice the skin tightness because of the very gradual and slow progress over a few years.

They may even look younger with the loss of wrinkles because of skin tightness.

Another early symptom is Raynaud's phenomenon, when the blood vessels go into spasm because of cold or emotional stress.

Typically, the fingers or toes turn pale white or bluish, then reddish as the body temperature warms up, and circulation returns to normal. There may be associated numbness or a sensation of pins and needles.

The nature of internal organ complications determines whether the patient will do well.

The gastrointestinal tract, heart, lungs, joints and muscles are the most frequent targets, but rarely the kidneys.

Early diagnosis and appropriate monitoring of patients with SSc are critical for getting the disease under control quickly.

Patients who have a very mild form of the disease do not need medications.

However, careful monitoring for symptoms such as breathlessness, together with heart and lung investigations, is needed to identify complications early.

Ms B was treated with immuno-suppressive drugs, which helped to reset the immune system so that it would stop attacking the patient's body.

Physiotherapy also helps to maintain joint mobility and prevent worsening contractures.

Within a few months of treatment, her skin softened and her joints gradually became less stiff. She walked more easily, in her own words, "as if on a cloud". Her positive attitude also helped in her recovery.

Today, she and other scleroderma patients have started a support group to raise awareness of the disease. They have launched the Sclerowarriorsg page on Facebook and are contributing to fund-raising efforts for the Reverie Rheumatology Research Fund.

We hope that research will continue to accelerate discoveries towards developing new treatments.

  • Dr Andrea Low is head and senior consultant in the department of rheumatology & immunology at Singapore General Hospital.
  • Scleroderma patients worldwide celebrate World Scleroderma Month in June. Tomorrow is World Scleroderma Day, in memory of Swiss artist Paul Klee who suffered from the condition and died on June 29, 1940.
A version of this article appeared in the print edition of The Straits Times on June 28, 2016, with the headline 'Softening a 'hard skin' condition'. Print Edition | Subscribe