Blue rubber bleb nevus syndrome associated with consumption coagulopathy: treatment with interferon


APAK H. , Celkan T. T. , OZKAN A. , YILDIZ i. , AYDEMIR E. H. , OZDIL S., ...More

DERMATOLOGY, vol.208, no.4, pp.345-348, 2004 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 208 Issue: 4
  • Publication Date: 2004
  • Doi Number: 10.1159/000077846
  • Title of Journal : DERMATOLOGY
  • Page Numbers: pp.345-348
  • Keywords: blue rubber bleb nevus syndrome, bean syndrome, disseminated intravascular coagulation, interferon, HEMANGIOMAS, INVOLVEMENT, NAEVUS

Abstract

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 beta. 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 beta in BRBNS might be beneficial. Copyright (C) 2004 S. Karger AG, Basel.