Bilateral periorbital ecchymoses. An often missed sign of amyloid purpura.

Détails

ID Serval
serval:BIB_E8C94D970BD1
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Institution
Titre
Bilateral periorbital ecchymoses. An often missed sign of amyloid purpura.
Périodique
Hämostaseologie
Auteur⸱e⸱s
Colucci G., Alberio L., Demarmels Biasiutti F., Lämmle B.
ISSN
0720-9355 (Print)
ISSN-L
0720-9355
Statut éditorial
Publié
Date de publication
2014
Peer-reviewed
Oui
Volume
34
Numéro
3
Pages
249-252
Langue
anglais
Notes
Publication types: Journal Article Publication Status: ppublish
Résumé
Immunoglobulin light chain (AL) amyloidosis is a systemic disease caused by a plasma cell clone synthesizing an unstable light chain, which forms amyloid fibrils. Deposition of amyloid fibrils affects primarily kidney, heart, nervous system, spleen, liver, gastrointestinal tract and the skin. Skin bleeding in these patients is called amyloid purpura. Classically, it occurs spontaneously and bilaterally in the periorbital region. Vessel wall fragility and damage by amyloid are the principal causes of periorbital and gastrointestinal bleeding. Additionally, coagulation factor inhibitory circulating paraprotein, hyperfibrinolysis, platelet dysfunction or isolated acquired factor X deficiency may contribute to even more severe, diffuse bleedings. Early diagnosis remains essential for improving prognosis of patients with AL amyloidosis. Although pictures of amyloid purpura have been often reported in the literature, the clinical diagnosis may be delayed. We report a case of cutaneous manifestation of AL amyloidosis diagnosed not until one year after the appearance of the first symptoms. Diagnostic work-up revealed that the patient suffered from multiple myeloma with secondary AL amyloidosis. Atraumatic ecchymoses at the face, particularly the eyelids as well as in the neck should raise the suspicion of AL amyloidosis.
Pubmed
Web of science
Création de la notice
19/09/2014 18:11
Dernière modification de la notice
20/08/2019 17:11
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