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Amyloid purpura

Amyloid purpura is a condition marked by bleeding under the skin (purpura) in some individuals withamyloidosis.[1] Its cause is unknown, butcoagulation defects caused byamyloid are thought to contribute.

Amyloid purpura
Purpura
SpecialtyDermatology

Presentation

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Amyloid purpura usually occurs above the nipple-line and is found in the webbing of the neck and in the face and eyelids.[1]

Cause

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The precise cause of amyloid purpura is unknown, but several mechanisms are thought to contribute.[2] One may be a decrease in the level of circulatingfactor X,[2] aclotting factor necessary forcoagulation. The proposed mechanism for this decrease in factor X is that circulating amyloid fibrils bind and inactivate factor X.[2] Another contributing factor may be enhancedfibrinolysis,[2] the breakdown ofclots.Subendothelial deposits of amyloid may weakenblood vessels and lead to the extravasation of blood.[2][3] Amyloid deposits in thegastrointestinal tract andliver may also play a role in the development of amyloid purpura.[2]

Diagnosis

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Treatment

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Epidemiology

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Amyloid purpura affects a minority of individuals with amyloidosis.[1] For example, purpura is present early in the disease in approximately 15% of patients withprimary systemic amyloidosis.[4]

References

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  1. ^abcEder L, Bitterman H (June 2007). "Image in clinical medicine. Amyloid purpura".N. Engl. J. Med.356 (23): 2406.doi:10.1056/NEJMicm061510.PMID 17554122.
  2. ^abcdefGamba G, Montani N, Anesi E, et al. (March 2000)."Clotting alterations in primary systemic amyloidosis"(PDF).Haematologica.85 (3):289–92.PMID 10702818.
  3. ^Amyloidosis, Immunoglobulin-Related ateMedicine
  4. ^Kyle RA, Gertz MA (January 1995). "Primary systemic amyloidosis: clinical and laboratory features in 474 cases".Semin. Hematol.32 (1):45–59.PMID 7878478.

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