There are two main types of systemic scleroderma (more commonly known as systemic sclerosis – SSc).
- Limited Cutaneous Systemic Sclerosis: (Limited SSc) Comes on gradually; affects only some areas of your skin, generally the hands and telangiectasia on the face; organs may also be affected, particularly the lungs in the form of pulmonary hypertension.
- Diffuse Cutaneous Systemic Sclerosis: (Diffuse SSc) Comes on suddenly; commonly affects more of the skin than limited cutaneous; the organs are also commonly affected, often the heart and lungs through fibrosis.
There is also Sine Sclerosis which is a rarer type of the condition. Those with sine sclerosis do not have any evidence of scleroderma on the skin, but are still affected in their organs.
Systemic Sclerosis used to be called CREST. CREST stands for Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasias. It is considered an outdated term for a few reasons. One being that he ‘E’, standing for esophagael dysfunction, doesn’t summarise the entirety of the digestive complications that many people who live with systemic sclerosis experience. In fact, because of this, the acronym that seems like such a useful diagnosis tool, could actually hinder diagnosis by GPs.
Another reason proves to be an even more worrying one. According to sclero.org, GPs were diagnosing ‘CREST’ but weren’t completing the diagnosis by determining whether it was Limited or Diffuse Scleroderma. This meant many patients were unaware of the extent of their condition. This led to the term ‘scleroderma’ to be the preferred diagnostic term in the hopes of encouraging further investigation into the patient’s condition.