Friday, May 4, 2007
Blue rubber bleb nevus syndrome
Blue rubber bleb nevus syndrome, also known as the Bean syndrome, is an uncommon neurocutaneous disorder where scattered, bluish, rubbery nevi occur on the body surface. It was named this because of the blueness of the nevi’s and the rubbery texture that the nevi’s had. It was first observed by Gascoyen in 1860 because a tumor/nevi was suffocating one of their patients. These skin lesions are noticeable at birth and continuously bleed and can occur in internal organs in the body. Symptoms of blue-rubber-bleb-nevus-system would include, clumsiness, dark purple nevi’s, and muscle weakness, bleeding, and anemia, however, these nevi’s would not be cancerous and can also vary in size, shape, color and number and can be sensitive to touch. It can occur in the “intestine, eyes, nasopharynx, parotids, lungs, liver, spleen, heart, brain, pleura, peritoneum, pericardium, skeletal muscles, bladder, and penis lesions” (http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0041-878120000001000060). The causes in this syndrome are unknown it mostly occurs in sporadic cases and is hard to be monitored and they will increase in size and number. This disease affects both males and women but it is rare in the black race. There have been 150 recorded cases and even in a family where there is no sign of the disease a child can still be affected by it.
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