What is it?
Scleroderma, or Systemic Sclerosis, is a chronic connective tissue dIsease generally classified as one of the autoimmune rheumatic diseases. The word “scleroderma” comes from two Greek words; “skleros” meaning hard and “derma” meaning skin.
Hardening of the skin is considered the hallmark of the disease. This hardening is caused by the overproduction of collagen which is laid down in the skin and internal organs resulting in scarring and reduced function of the affected organs.
Scleroderma is NOT contagious, infectious, cancerous or malignant.
The symptoms of Scleroderma vary greatly for each person with the affects ranging from mild to life threatening.
Symptoms may include:
Thickening of the skin causing a tight and shiny appearance
Heartburn or Reflux
Difficulty in swallowing
Increased sensitivity to cold in the hands and feet (Raynaud’s Phenomenon)
Appearance of tiny blood vessels on the skin (Telangiectasia)
Difficulties in breathing or shortness of breath
Who does Scleroderma affect?
The number of people suffering from Scleroderma in Australia varies from source to source, although it is widely accepted that this number is between 5000 and 10000. Overall female patients out-number male patients by 4 to 1 with onset of the disease generally being most prevalent between the ages of 25 and 55. At present there is no conclusive evidence to suggest that Scleroderma is hereditary.
What causes Scleroderma?
As the precise cause of Scleroderma has not yet been determined, there is currently no cure for the disease. Treatment consists of maintenance of the symptoms with the view to slowing disease progression and improvement in the quality of life for the patient.
Localised Scleroderma affects the skin, related tissues and sometimes the muscle below. The internal organs are not involved and this form of the disease cannot progress into systemic sclerosis. Children are more likely to develop localised scleroderma than adults.
Types of Scleroderma
Scleroderma, or Systemic Sclerosis, can be broadly classified into two groups; localised and systemic.
In Systemic Scleroderma the changes which occur may affect the connective tissues in many parts of the body. Areas of involvement may include the skin, oesophagus, gastrointestinal tract, lungs, heart, kidneys and other internal organs as well as blood vessels, muscles and joints.
The names scleroderma and systemic sclerosis are interchangeable. Increasingly the term “systemic sclerosis” is being used as it more adequately describes the disease.
Limited Systemic Sclerosis
Morphea is characterised by thickened patches of skin which are oval in shape. These patches may grow larger or shrink and often disappear over a period of time.
As the name suggests, this form of scleroderma usually develops as a line of thickened skin on an arm or leg and sometimes on the forehead. Linear scleroderma differs from Morphea in that it also affects the deeper layers of skin with the mobility of underlying joints sometimes being involved.
Limited Systemic Sclerosis tends to develop gradually over a number of years and is generally less severe than Diffuse Scleroderma. It is often referred to as CREST, an acronym for the following:
Calcinosis- calcium deposits under the skin
Raynaud's phenomenon- spasms in the small blood vessels of the hands and/or feet in response to cold or anxiety.
Esophageal dysfunction- in the upper esophagus causing swallowing difficulties and in the lower esophagus causing chronic heartburn.
Sclerodactyly- thickened skin on the fingers caused by excess collagen resulting in an inability to bend or straighten the fingers.
Telangiectasia- small broken blood vessels on the surface of the skin.
Diffuse Systemic Sclerosis
In contrast to the limited form, Diffuse Systemic Sclerosis usually develops suddenly with a larger part of the body being affected. The damage to internal organs tends to be more severe with the long term outlook being the most serious of all the forms of this disease.