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
em En
| WPRIM
| ID: wpr-155337
Biblioteca responsável:
WPRO
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.
Palavras-chave
Texto completo:
1
Índice:
WPRIM
Assunto principal:
Pele
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Células-Tronco
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Trombocitose
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Doenças de von Willebrand
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Medula Óssea
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Anticorpos Antifosfolipídeos
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Eritromelalgia
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Livedo Reticular
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Trombocitemia Essencial
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Anticorpos
Limite:
Humans
Idioma:
En
Revista:
Annals of Dermatology
Ano de publicação:
2012
Tipo de documento:
Article