blue rubber bubble nevus syndrome
Introduction
Introduction to blue rubber blister syndrome BlueRubberBlebNevusSyndrome is a spongy or capillary hemangioma that exists in both the skin and the gastrointestinal tract. It was first described by Bean in 1958, so it is called Bean Syndrome. Intrinsic is caused by embryonic differentiation and developmental disorders, and is an autosomal dominant hereditary disease. Clinically, patients often have hematemesis, blood in the stool and rectal bleeding, resulting in anemia, skin veins dilated, like a prominent, purple-red or blue mass, soft, small lesions can be faded after compression, local pain or touch Pain, sweating above the mass. basic knowledge Sickness ratio: 0.5% Susceptible people: no specific population Mode of infection: non-infectious Complications: anemia
Cause
Causes of blue rubber blister syndrome
There are 3 cases of pathological changes in the skin or digestive tract:
1. Large cavernous hemangioma replaces normal tissue, affects vital organs and blocks tubular structures such as the intestines, trachea, etc.
2. The blood sac is a blue rubber-like nipple that covers the thin layer of skin.
3. Irregular blue spots, sometimes punctate, invading the surrounding normal skin, and thus various pigment changes from blue-black, blue, light blue to normal skin color, histological examination as capillary or spongy Hemangiomas, there are smooth muscle fibers between the walls of the tube, and there are most sweat glands near the blood vessels.
Prevention
Blue Scrub Syndrome Prevention
The cause of this disease is not clear, and it is related to autosomal recessive inheritance. It is usually related to the marriage of a close relative, and the disease cannot be directly prevented. For patients with a family history of suspected chromosomal abnormalities, genetic screening should be carried out to avoid the offspring of the disease caused by chromosome inheritance after marriage. At the same time, attention should also be paid to strengthening nutrition during pregnancy, reasonable diet, and avoiding emotional stimuli that affect embryonic development.
Complication
Blue rubber blister syndrome complications Complications anemia
1. Anemia: Blood tests for red blood cells and hemoglobin levels below 10% of normal levels, adults generally have red blood cell counts of <4.0×1012/L, women <3.5×1012/L; hemoglobin males <120g.
2. Toxemia, also known as "multiple abscesses": In the past, it was called "septic sepsis", which is one of the most serious systemic suppurative infections. A large number of bacteria in a group form a bacterial embolus. When the bacterial emboli intermittently invades the human blood circulation and stays somewhere in the body, causing a series of symptoms, it is called sepsis. The most common pathogens are Staphylococcus aureus and Staphylococcus epidermidis, and the clinically prominent manifestation is the formation of multiple abscesses.
Symptom
Blue rubber blister syndrome symptoms common symptoms blood in the stool
Clinically, patients often have hematemesis, blood in the stool and rectal bleeding, resulting in anemia, skin veins dilated, like a prominent, purple-red or blue mass, soft, small lesions can be faded after compression, local pain or touch Pain, sweating above the mass.
Examine
Blue blisters syndrome check
Do an angiographic examination. Angiography is the injection of a liquid angiographic contrast agent into a blood vessel, which is then irradiated with an angiograph. Iodine contrast agents are the most commonly used contrast agents for angiography, but iodine contrast agents are not available for angiography in patients who are allergic to iodine contrast agents or have poor kidney function. Angiography refers to the introduction of a contrast agent into a target blood vessel to visualize the blood vessel of interest for diagnostic purposes.
Diagnosis
Diagnosis of blue rubber blister syndrome
diagnosis
When diagnosed, anyone with special skin manifestations such as blue spots and gastrointestinal bleeding should think of this disease.
Differential diagnosis
It should be differentiated from other diseases of cutaneous hemangioma associated with gastrointestinal hemangioma, such as hereditary hemorrhagic telangiectasia (Osler's disease), multiple localized hemangioma, Klippel-Weber syndrome (wine-colored hemangioma) And systemic hemangioma and the like.
