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Blue Rubber Bleb Nevus Syndrome
Author(s) -
Oranje Arnold P.
Publication year - 1986
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1986.tb00530.x
Subject(s) - medicine , dermatology , coagulopathy , nevus , vascular disease , hemangioma , gastrointestinal tract , anemia , surgery , melanoma , cancer research
Blue rubber bleb nevus syndrome (BRBN) is a rare disease entity of which at least 22 cases of pediatric origin have been described since 1958. Only a few known cases have developed in adulthood. This syndrome is characterized by cutaneous, usually multiple, cavernous hemangiomas associated with similar lesions of the gastrointestinal tract. The cutaneous hemangiomas are a variant of the cavernous type, which are generally soft, rubbery, and compressible. Most cases are sporadic, although autosomal dominant inheritance has been described. Complications of this syndrome may include gastrointestinal bleeding leading to anemia, amputations of extremities, and hematologic disturbances such as chronic consumption coagulopathy. Children suffering from BRBN syndrome should be surveyed extensively for comprehensive care. This syndrome is important to consider in cases of unexplained intestinal bleeding. Therapy is mainly symptomatic and directed to complications.