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Related Concept Videos

Amyloid Fibrils03:03

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Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining,...
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Performing and Processing FNA of Anterior Fat Pad for Amyloid
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Primary localised cutaneous amyloidosis--a systematic review.

Britta Kaltoft1, Grethe Schmidt, Anne Falensteen Lauritzen

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Primary localised cutaneous amyloidosis (PLCA) is typically benign with a good prognosis. This review found a low risk of systemic amyloidosis (SA) and local recurrence for PLCA patients.

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Area of Science:

  • Dermatology
  • Oncology
  • Pathology

Background:

  • Amyloidosis involves extracellular deposits of misfolded proteins (amyloid fibrils) in tissues.
  • Primary localised cutaneous amyloidosis (PLCA) is a specific form affecting the skin.
  • Systemic amyloidosis (SA) represents a more widespread form of the disease.

Purpose of the Study:

  • To evaluate the risk of developing systemic amyloidosis (SA) from primary localised cutaneous amyloidosis (PLCA).
  • To assess the risk of local recurrence in patients with PLCA.
  • To compare treatment methods against the risk of local recurrence.

Main Methods:

  • A comprehensive literature search was conducted.
  • 77 articles on PLCA were identified, with 23 excluded, resulting in 54 included studies.
  • Data from 94 patients were analyzed.

Main Results:

  • The majority of PLCA cases (69%) were the nodular subtype.
  • Local recurrence occurred in 9% of patients, primarily in males with nodular PLCA on the face.
  • Only 1% of patients developed systemic amyloidosis (SA).

Conclusions:

  • PLCA is a benign condition with a favorable prognosis.
  • The risk of developing SA from PLCA is low (1%).
  • The risk of local recurrence or new lesions is 9%, with no significant difference based on primary treatment.