Sinus pericranii and venous angioma in the blue-rubber bleb nevus syndrome.

The pathologic changes of blue-rubber bleb nevus syndrome have been known for over 100 years, but it was not until 1958 that a detailed description of the syndrome was published. Bean [1] described a disorder characterized by bluish nevi scattered throughout the skin and angiomas of the gastrointestinal tract that could be a source of serious bleeding. Larger skin lesions typically have the consistency of rubber nipples, have a bluish cast, are easily compressible, and refill promptly when compression is released . The syndrome is well known among dermatologists and gastroenterologists but generally not known among neuroradiologists. We draw attention to this syndrome because, although rare, it seems to be associated with significant central nervous system (eNS) abnormalities. We recently evaluated a patient with this syndrome who had frontal sinus pericranii and a giant venous angioma that drained extracranially through a large varix.