Nevus

Nevus is a rare disorder that consists groups of abnormal blood vessels (venous malformations) affecting the skin and gastrointestinal (GI) tract. G. Gascoyen reported the first case of the abnormal blood vessels in 1862. In 1958, W. Bean named the characteristic vessels "blue rubber bleb " nevi (BRBN) after their color, texture, and bladder-like appearance. For this reason, BRBNS has been also referred to as Bean's syndrome. It is commonly associated with GI bleeding and anemia (low blood count). BRBNS is not a "virus", but a condition that you are born with "congenital."

Rubbery venous malformations usually become apparent by age 2, and last for the entire life, but do not become cancerous. The venous malformations can be found on the skin from head to toe. They can also be found in many internal organs like the liver, spleen, lungs, and the intestines

There are no prevention guidelines for this syndrome as it is an inherited disease. Several authors have suggested various modes of inheritance. Berlyne et al (1960) described transmission through five generations with males and females affected equally, suggesting an autosomal dominant inheritance. Talbot et al. (1970) described a family where only males were affected. Walshe et al. (1966) and Munkvad (1983) suggested autosomal dominance in three case reports. The later three studies did not document gastrointestinal lesions or bleeding.

The cause of BRBNS is unknown. Most cases are sporadic, but autosomal dominance has been reported. The disorder has not been localized to a specific chromosome or gene defect. The venous malformations in the intestines have a tendency to bleed causing anemia. The bleeding can be either slow or brisk. If bleeding is slow, anemia may be corrected or prevented by iron supplementation (oral or intravenous). More rapid blood loss may require surgery to treat the venous malformations and/or blood transfusions. Large lesions can block the airway and may require tracheostomy. Large lesions in the extremities may be painful and may require disfiguring surgery or even amputation. Patients may also have intestinal obstruction due to the intestinal lesions.

Diagnosis is based on following manifestations: 

Cutaneous manifestations: Skin lesions are usually highly characteristic, as multiple, protuberant, dark blue, compressible blebs, a few millimeters to several centimeters in diameter and varied in hue and shape. They have the look and feel of a rubber nipple. Lesions may be few in number or range into the hundreds. Three types of cutaneous lesions have been described: (1) blue, rubbery, blood-filled sacs with a smooth or wrinkled surface that are easily compressible and promptly refill when pressure is released, (2) large, disfiguring, cavernous lesions that may compress vital structures and (3) blue, irregular macules. The color of cavernous lesions may appear red, purple-red, blue or black, and morphology varies from flat to elevated, occasionally pedunculated, nodules. Lesions may be tender to palpation, and overlying hyperhidrosis may be noted. Skin lesions rarely bleed unless traumatized. Progression in size and number of blebs may occur with advancing age. The lesions are principally located on the upper limbs, trunk and perineum, but may occur anywhere.

Extracutaneous manifestations: The visceral organ system most commonly affected is the gastrointestinal tract. Vascular malformations may occur anywhere from oral to anal mucosa but predominantly occur in the small bowel.

Orthopedic manifestations: These include skeletal bowing, pathologic fractures, bony overgrowth and articular derangement. Bone deformities may arise as a result of pressure effects from adjacent vascular lesions. Vertebral lesions have caused spinal cord compression and vertebral collapse, and the lesions may extend into joint spaces affecting range of motion. Extensive lesions have also been reported on the feet, impairing ambulation. Debilitating enlargement occasionally necessitates amputation of the affected limb.
Blue rubber bleb nevi have been reported in the skull, central nervous system, thyroid, parotid, eyes, oral cavity, lungs, pleura, pericardium, musculoskeletal system, peritoneal cavity, mesentary, kidney, liver, spleen, penis, vulva and bladder.

Eat fruits and vegetables and concentrate on light foods. Avoid fats and red meat. Limit caffeine intake and stop smoking.

It is vital that smoking and caffeine are avoided and a healthy routine is adopted.

It is an inherited condition but still one should try to concentrate on maintaining a relaxed state of mind and body as that helps in keeping off any disease.