Systemic sclerosis

What it is

Systemic sclerosis is a systemic autoimmune disease characterized by vasculopathy and fibrosis of the skin and other organs. It comes from abnormal growth of connective tissue, which leads to diffuse thickening and hardening of the skin and of the inner organs, so the skin is tight and has lost its normal fold. Based on the extent of cutaneous involvement it is categorized as limited or diffuse.

Written short, scleroderma is an autoimmune, inflammatory, fibrotic CT disease, and what it means at the skin is a thickened sclerotic skin lesion. It is to be considered the most common cause of a Raynaud phenomenon that is not primary, and it may cause a low T4.

How it is classified

The classification is in two, localized and systemic.

Localized:

• Linear: mostly in childhood, and it occurs in a dermatomal distribution
• Morphoea: patches of sclerotic skin

Systemic sclerosis, which is skin plus internal organ:

• Limited cutaneous: limited to hand and face, plus CREST
• Diffuse cutaneous: more proximal skin and trunk, and more cardiac, lung and renal
• SS sine scleroderma
• Environmental