AL amyloidosis

A plasma cell disorder characterized by the aggregation and deposition of insoluble amyloid fibrils derived from misfolding of monoclonal immunoglobulin light chains usually produced by a plasma cell tumor. It usually presents as primary systemic amyloidosis (PSA) with multiple organ involvement and less frequently as primary localized amyloidosis (PLA) restricted to a single organ.

Periorbital purpura

Multiple red/purple spots on the skin that surrounds the eyes that do not blanch (whiten) upon pressure. Purpura is caused by subcutaneous bleeding.


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PMID (PMCID)
26480258
FEMALE
[Periorbital purpura: a pathognomonic but late sign of AL amyloidosis].
Visconti L, Cernaro V, Ferrara D, Lacava V, Ricciardi CA, Di Mauro E, Amico L, Santoro D, Buemi M.
G Ital Nefrol. 2015;32(5):.
Periorbital purpura is a pathognomonic sign of AL amyloidosis but it appears late.