1. Briefly describe the clinical features and pathology of systemic sclerosis[]
Clinical Features[]
Scleroderma presents in two distinctive categories, the clinical features of which are listed here.
Pathology[]
The skin in scleroderma initially shows oedema, and then sclerosis and hardening (induration). The affected skin exhibits
- A marked increase in collagen fibres in the reticular dermis
- Thinning of the epidermis with loss of rete ridges
- Atrophy of dermal appendages
- hyalinisation
- Arteriole obliteration
- Varied mononuclear infiltration - primarily of T-cells
Indurated skin may progress to atrophy or revert back to normal. Collagen increase may also occur in synovium, lungs, GI tract, heart and kidneys.
Lesions in the arteries, arterioles and capillaries are typical, and in some cases may be the first demonstrable pathologic finding in the disease. Initial subintimal oedema with fibrin deposition is followed by thickening and fibrosis of the vessel and reduplication or fraying of the elastic lamina. The vessels can become severely restricted in terms of blood flow, and may become occluded by thromboses. Organ systems can become fibrosed and necrosed.