Localised Amyloidosis

Localised Amyloidosis Syndromes

Amyloidosis is usually a systemic disease. This means that many body organs and systems may be affected. Amyloidosis may also be ‘local’ or ‘localised’ which means that just a single organ or part of the body is affected. About 12% of all newly diagnosed amyloidosis patients at the National Amyloidosis Centre have localised amyloidosis.In these rare conditions, amyloid deposits are found in just one part of the body. They almost never progress to systemic amyloidosis.

It is usually possible to cure these problems by surgical excision of the deposits.

Foci of abnormal plasma cells producing AL amyloid deposits limited to just one place may be present in the:

  • skin
  • upper airways and respiratory tract
  • genital and urinary system
  • eye (usually affecting the conjunctiva)
  • lymph nodes (rare)
  • gastrointestinal tract (rare)

Localised AL amyloidosis is not usually associated with detection of a serum monoclonal protein/paraprotein in blood tests.

Other types of localised amyloid may affect the:

  1. Skin:
  • Macular cutaneous amyloidosis: amyloid fibrils are derived from a skin protein called galactin. There is an itchy rash, with scaly, brownish lesions that may be flat or raised (macules or papules). It is common in people with dark skin.
  • Hereditary cutaneous amyloid: rare, with unknown fibril type, sometimes with an associated disease affecting many body systems.

2.  Eye: localised amyloid typically affecting the conjunctiva is usually of AL type. This is treated by local surgical measures.

For more information on localised amyloidosis, see our patient information leaflet.