The Bean syndrome is a very rare condition that affects very few people around the world. In addition, Bean Syndrome or BRBNS is a rare syndrome characterized by cutaneous and visceral venous malformations, mainly affecting the gastrointestinal tract.
It was first described by Gascoyen in 1860, however, William B. Bean coined the term “blue rubber bleb nevus syndrome” in 1958. 6 Recently, Rudolf Happle proposed the name “rubber bleb angiomatosis”. blue ”“ In 2010, referring to the type of malformations it presents.
The nature of vascular malformations is venous, and it is important to mention that Bean’s original description, and even multiple recent publications, have contributed to confusion about the real nature of vascular lesions occurring in this condition, as they refer to them as “ hemangiomas ”, probably applying the previous nomenclature that used the term“ cavernous hemangioma ”to refer to vascular venous malformations.
What is Bean Syndrome
The Bean syndrome or Blue Nevus Syndrome or SNA (BRBNS or Blue nebulos rubber) is a rare disease of the skin , potentially fatal, but easily diagnosed.
It was first described in 1860, the English dermatologist George Gaskoin, but is named for William Bennett Bean, who documented it most extensively in 1958.
It is an upper trunk skin syndrome typical of chronic liver failure. It is characterized by the appearance of one or more blue nodules very susceptible to fatal bleeding. It is associated with digestive tract hemangiomas.
Are complications associated gastrointestinal bleeding, causing deficiency anemia iron , amputation of limbs, eye injury, intussusception and diseases orthopedic. Nasal pain and regional hyperhidrosis may occur. The etiology is unknown.
It can be observed in both sexes during early childhood. They may appear from one to a hundred small subcutaneous nodules of rubber-like consistency, which render prominence in the form of small polyps accompanied by recurring vanes and pallidomucous pallor.
Bean Syndrome Symptoms
Sporadic Night Pain
Subcutaneous nodules (small polyps)
Bean Syndrome Treatment
The treatment of skin lesions is indicated for cosmetic purposes only or when they represent a functional problem. As therapeutic options, ruby, argon and carbon dioxide laser, electrodesication, surgical excision and sclerotherapy were proposed; However, the pulsed light laser seems to be most effective in removing multiple lesions without recurrence.
In relation to gastrointestinal lesions, in cases where there is occult or evident blood loss, oral iron supplementation should be indicated and, when conditioning hemodynamic decompensation, blood transfusion.
For gastrointestinal lesions with active bleeding proposed treatment with endoscopic photocoagulation, Nd: YAG laser, sclerotherapy, use of octreotide acid ε-aminocaproic acid and acid nadolol, surgical excision and bowel resection, the latter in patients with massive hemorrhage, lesions or intussusception intestinal.
The use of interferon, steroids or both has been proposed as a therapeutic option in patients with skin bleeding and gastrointestinal lesions; However, even when they are effective in achieving disease stabilization , and even partial remission of lesions, after discontinuation of treatment, lesions return to their initial characteristics; The authors who propose this feature mention that although there is no other more effective treatment, this may be a useful alternative in cases with important conditions, as is our patient.
Orthopedic changes may require physical therapy, serial plaster bandages for posture correction, braces, surgical scission and, in extreme cases, amputation.
The rest of the affected organs should be treated if there is evidence of bleeding from the lesions and sequelae, particularly at the central nervous system level, physiotherapy and rehabilitation.