About blue rubber bleb nevus
What is blue rubber bleb nevus?
Blue rubber bleb nevus syndrome (BRBNS) is a rare blood vessel (vascular) disorder that affects the skin and internal organs of the body. Multiple distinctive skin lesions are usually characteristic of this disorder and are often present at birth or present during early childhood. Lesions in the gastrointestinal tract frequently become apparent during childhood or early adulthood. The lesions are multifocal venous malformations, resulting from abnormal embryonic blood vessel development.
What are the symptoms for blue rubber bleb nevus?
Multiple venous malformations in various organ systems symptom was found in the blue rubber bleb nevus condition
BRBNS is characterized by soft, elevated Lesions on the skin or just under the skin that are dark blue, red, purple-red or in color. The venous malformations may be tender, contain blood and be easily compressed and are usually located on the upper limbs, trunk and soles of the feet but can occur anywhere. The Lesions increase in size and become more apparent over time but have not been reported to become cancerous. The organ system most commonly affected by BRBNS is the gastrointestinal (GI) tract, particularly the small intestine. The Lesions in the GI tract often bleed and can lead to mild or severe anemia. Iron replacement and/or frequent blood transfusions may be required. The GI Lesions can also cause an obstruction or blockage (intussusception) of part of the bowel. Skeletal abnormalities and venous malformations in muscle are sometimes associated with BRBNS.
What are the causes for blue rubber bleb nevus?
Blue rubber bleb nevus syndrome is sporadic. Although families have been described in which the condition follows autosomal dominant inheritance, these families actually have other multifocal venous malformations.
What are the treatments for blue rubber bleb nevus?
Iron therapy and blood transfusions are used to conservatively manage BRBNS. The skin lesions associated with BRBNS can be treated with laser therapy, injection of chemicals that collapse the lesion (sclerotherapy) or surgical removal. Lesions in the gastrointestinal system are usually not removed unless bleeding leads to anemia and necessitates repeated blood transfusions. Gastrointestinal lesions can be safely removed surgically, but one or several lengthy operations may be required.
What are the risk factors for blue rubber bleb nevus?
Blue rubber bleb nevus syndrome is also known as Bean syndrome. It is a rare syndrome of venous malformations that arise in the skin and gastrointestinal tract.
Risk factors.
- Since the disease is a genetic disorder, there are no risk factors associated with this disease. However, consanguineous marriage is a leading cause of disease transmission.
- Some patients may have mutations in genetic levels, which can affect the person.
- Since a person is already suffering from gastrointestinal malformations, other lifestyle conditions can also be necessary risk factors
- this disease can affect both genders equally.
- This disease is much more severe in younger patients and often get severe during adolescence.
Common treatments include
- Blood transfusions and iron supplementation to treat anemia caused by gastrointestinal bleeding
- Sclerotherapy, where doctors inject a medicine called a sclerosant into an abnormal vessel, causing the vessel to clot and shrink
- Surgery to remove painful skin blebs or gastrointestinal blebs causing significant bleeding
Symptoms
Soft, elevated lesions on the skin or just under the skin that are dark blue, red, purple-red or in color,Skeletal abnormalities and venous malformations in muscle,Multiple venous malformations in various organ systems, including the liver, spleen, heart, eye, and central nervous system
Conditions
A rare syndrome of venous malformations that arise in the skin and gastrointestinal tract,Skeletal abnormalities
Drugs
Iron therapy,Blood transfusions,Laser therapy
Is there a cure/medications for blue rubber bleb nevus?
Blue rubber bled nervous syndrome, also known as bean syndrome. It is a rare syndrome of vascular malformation that results in abnormal growth of veins that may stretch and grow over time.
- This syndrome arises on the skin and gastrointestinal tract. However, many people have vascular malformation across various organs like the liver, spleen, eye, heart, etc.
- This bean syndrome causes cosmetic problems and pain, and if present on the bottom of the feet, it may result in difficulty walking.
- A person with this syndrome has a highly significant risk of gastrointestinal and severe iron deficiency.
- The medical management of this syndrome requires the assistance of hematology, dermatology, gastroenterology, and other department related to this field of expertise.
- Unformataley, Blue rubber bled nervous syndrome has no cure, but it can be treated through the following steps.
- The treatment is not required for the skin spots, but a person can undergo laser surgery for cosmetic reasons or if the syndrome is causing problems in the affected area.
- This syndrome often causes Cutaneous syndrome that can be effectively treated by surgery, laser, sclerotherapy, cryotherapy, and electrodesiccation.
- A person may lose a lot of blood from Gastrointestinal bleeding, in order to solve this complication, iron supplements and blood transfusion are prescribed to patients.
Symptoms
Soft, elevated lesions on the skin or just under the skin that are dark blue, red, purple-red or in color,Skeletal abnormalities and venous malformations in muscle,Multiple venous malformations in various organ systems, including the liver, spleen, heart, eye, and central nervous system
Conditions
A rare syndrome of venous malformations that arise in the skin and gastrointestinal tract,Skeletal abnormalities
Drugs
Iron therapy,Blood transfusions,Laser therapy
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