Document Detail

Venous malformations in blue rubber bleb nevus syndrome: variable onset of presentation.
MedLine Citation:
PMID:  15097941     Owner:  NLM     Status:  MEDLINE    
Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder characterized by discrete venous malformations of varying size and appearance that are present on the skin and within the gastrointestinal tract. The characteristic cutaneous lesions consist of deep-blue, soft, rubbery blebs, which are easily compressible. A serious complication is gastrointestinal bleeding. Because venous malformations were described historically as cavernous hemangiomas, the lesions of BRBNS were also inappropriately called hemangiomas in the literature. We describe 3 cases to delineate the venous malformations of BRBNS and to highlight their variable onset of presentation and progression. In one case, a venous malformation was noted during a prenatal ultrasound evaluation at 5 months gestation. The other 2 cases demonstrated a lack of the classic cutaneous lesions at birth. BRBNS consists of multiple venous malformations, rather than hemangiomas as described. Subcutaneous venous malformations may occasionally be the sole presenting finding in patients with this unusual syndrome, and may be evident even in the prenatal period.
Walter K Nahm; Sharon Moise; Lawrence F Eichenfield; Amy S Paller; Laura Nathanson; Denise M Malicki; Sheila Fallon Friedlander
Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Journal of the American Academy of Dermatology     Volume:  50     ISSN:  0190-9622     ISO Abbreviation:  J. Am. Acad. Dermatol.     Publication Date:  2004 May 
Date Detail:
Created Date:  2004-04-20     Completed Date:  2004-06-04     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  7907132     Medline TA:  J Am Acad Dermatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  S101-6     Citation Subset:  IM    
Division of Dermatology, University of California-San Diego School of Medicine, San Diego, CA 92113, USA.
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MeSH Terms
Gastrointestinal Neoplasms / congenital,  pathology
Infant, Newborn
Nevus, Blue / blood supply*,  congenital
Skin / blood supply
Skin Neoplasms / blood supply*,  congenital
Ultrasonography, Prenatal
Veins / abnormalities*

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