Erythromelalgia and Livedo Reticularis in a Patient with Essential Thrombocythemia, Acquired von Willebrand Disease, and Elevated Anti-Phospholipid Antibodies
Annals of Dermatology
;
: 214-217, 2012.
Article
Dans Anglais
| WPRIM
| ID: wpr-155337
ABSTRACT
Essential thrombocythemia (ET) is a clonal stem cell disease characterized by isolated thrombocytosis and thrombohemorrhagic complications. We describe an unusual case of ET primarly presenting with skin symptoms including erythromelalgia and livedo reticularis (racemosa-type). Persistent thrombocytosis, bone marrow findings, JAK2 gene mutation, and markedly decreased ristocetin-cofactor activity were consistent with the diagnosis of ET and acquired von Willebrand disease. Elevated antiphospholipid antibodies were also found. The present case highlights the complex nature and diagnostic challenge of myeloproliferative disorders such as ET, which can involve multiple organ systems and often shows a variety of microvascular complications, coagulation anomalies, and autoimmune phenomena.
Texte intégral:
Disponible
Indice:
WPRIM (Pacifique occidental)
Sujet Principal:
Peau
/
Cellules souches
/
Thrombocytose
/
Maladies de von Willebrand
/
Moelle osseuse
/
Anticorps antiphospholipides
/
Érythromélalgie
/
Livedo réticulaire
/
Thrombocytémie essentielle
/
Anticorps
Limites du sujet:
Humains
langue:
Anglais
Texte intégral:
Annals of Dermatology
Année:
2012
Type:
Article
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