Blue Rubber Bleb Nevus Syndrome Associated with Consumption Coagulopathy: Treatment with Interferon

The blue rubber bleb nevus syndrome (BRBNS) is a rare vascular malformation syndrome with cutaneous and visceral lesions frequently associated with serious, even fatal bleeding. No systemic therapy is currently available. We report here a case with disseminated skin and gastrointestinal venous malfo...

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Veröffentlicht in:Dermatology (Basel) 2004-01, Vol.208 (4), p.345-348
Hauptverfasser: Apak, H., Celkan, T., Özkan, A., Yildiz, I., Aydemir, E.H., Ozdil, S., Kuruoglu, S.
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Sprache:eng
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Zusammenfassung:The blue rubber bleb nevus syndrome (BRBNS) is a rare vascular malformation syndrome with cutaneous and visceral lesions frequently associated with serious, even fatal bleeding. No systemic therapy is currently available. We report here a case with disseminated skin and gastrointestinal venous malformations and findings of disseminated intravascular coagulation that was treated with interferon β. The disseminated intravascular coagulation manifestations were cleared with the treatment. The regression of the coagulopathy in our patient led us to think that the use of interferon β in BRBNS might be beneficial.
ISSN:1018-8665
1421-9832
DOI:10.1159/000077846