What are the different forms of Scleroderma?

There are two main forms of Scleroderma: Localised Scleroderma and Systemic Scleroderma (or Systemic Sclerosis). The systemic form is more extensive disease often involving multiple systems.

1. Localised Scleroderma involves the skin and soft tissues. It does not progress to the systemic form and the involvement of internal organs is limited.

Localised scleroderma further divides into:

• morphea: thickened bands of hard skin (the patches are initially reddish, but later become whitish, often with a whitish or lilac edge); internal organs are not affected.
• linear scleroderma:skin disease as well as involvement of subcutaneous tissue, muscle and bone. In “coup de sabre” form, the scarring on the forehead resembles a sword stroke, hence the name; and often presents in childhood. Patients may have seizures, headaches, trigeminal neuralgia, muscle weakness, hemiparesis and visual changes.

2. Systemic Scleroderma (Systemic Sclerosis)

Underlying tissues and the major systems (or organs) and blood vessels are affected. This is the more severe due to multi-system involvement. The systemic form of the disease has two major groups, limited and diffuse.

• Limited cutaneous systemic sclerosis (lcSSc) (60% of cases)

Previously called CREST Syndrome, this is an acronym to describe the following clinical features:

C...Calcinosis - deposits of calcium in the skin

R...Raynaud's phenomenon affecting circulation to the extremities

E...Oesophageal dysfunction, characterised by poorly functioning muscle of the lower two-thirds of the oesophagus

S...Sclerodactyly refers to the localised thickening and tightness of the skin and tendons of the fingers or toes. This can give them a "shiny" and slightly puffy appearance, and can result in flexion deformities

T...Telangiectasias are tiny red spots, frequently on the face, hands and in the mouth behind the lips, due to dilated capillaries

• Diffuse cutaneous systemic sclerosis (dcSSc) (40% of cases)
• there is a short early phase of the disease with a general feeling of being ill, rapid involvement and progression of skin disease, as well as kidney, lung, heart and gastrointestinal involvement. The internal organs can be affected in both limited and diffuse systemic sclerosis (described below).
• Systemic sclerosis sine scleroderma(rare) – systemic features but skin is not involved.

Classification of Scleroderma

Amended November 2008