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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 27  |  Issue : 2  |  Page : 327-330

Mucocutaneous Bean syndrome: Review of literature and case report


1 Department of Oral Medicine and Radiology, Noorul Islam Dental College, Trivandrum, Kerala, India
2 Department of Oral and Maxillofacial Pathology, Pushpagiri College of Dental Sciences, Thiruvalla, Kerala, India

Date of Submission09-Apr-2015
Date of Acceptance22-Oct-2015
Date of Web Publication21-Nov-2015

Correspondence Address:
Devi Gopakumar
Department of Oral Medicine and Radiology, Noorul Islam Dental College, Trivandrum, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.170180

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   Abstract 

Bean syndrome or blue rubber bleb syndrome is a rare disorder of vascular channels with mucocutaneous manifestations. The clinical severity varies among patients from being totally symptom free to fatal bleeding resulting in anemia. As the histopathologic findings are not pathognomonic, diagnosis is usually made from the clinical presentation, though the imaging techniques reveal extension of the lesion. We report an asymptomatic case of blue rubber bleb syndrome who reported for dental treatment with oral and cutaneous manifestations.

Keywords: Angiomatosis, bleb, mucocutaneous, syndrome


How to cite this article:
Gopakumar D, Nair SS. Mucocutaneous Bean syndrome: Review of literature and case report. J Indian Acad Oral Med Radiol 2015;27:327-30

How to cite this URL:
Gopakumar D, Nair SS. Mucocutaneous Bean syndrome: Review of literature and case report. J Indian Acad Oral Med Radiol [serial online] 2015 [cited 2019 Jul 20];27:327-30. Available from: http://www.jiaomr.in/text.asp?2015/27/2/327/170180


   Introduction Top


Bean syndrome or blue rubber bleb nevus syndrome (BRBNS) or "blue rubber bleb syndrome" or "blue rubber-bleb nevus" is a rare disorder that consists mainly of abnormal blood vessels affecting the cutaneous and orogastrointestinal tract. [1],[2],[3] It is a rare angiomatosis condition with an estimated incidence of 1:14,000 births. [1],[4],[5] This syndrome is characterized by the association of cutaneous and gastrointestinal (GI) venous malformations with iron-deficiency anemia or GI bleeding. [6],[7] The disease was described by Gascoyen in 1860. [1],[7] In 1958, Bean differentiated this type of angiomatosis from other cutaneous vascular diseases. [3],[8] BRBNS can affect many organs and there are only fewer than 200 cases published. [6],[9],[10] This disease affects both adults and children without sex predilection, but is rare among black subjects. [1],[7],[8] In most cases, it is not possible to identify a family history, although a dominant pattern of inheritance has been reported. [2],[5],[10] We attempt a short review of literature and report a case of Bean syndrome.


   Case Report Top


A 56-year-old male came for routine dental checkup. On intraoral examination, multiple lesions, mostly soft, compressible, dark-blue papules, plaques, nodules, and macules were noticed in the oral cavity, mainly in the tongue, buccal mucosa, and soft palate [Figure 1] [Figure 2] [Figure 3] [Figure 4] [Figure 5]. Extraoral examination revealed multiple cutaneous nodular lesions distributed over the trunk and abdomen [Figure 6] and [Figure 7]. Patient had noticed these lesions from childhood. He described the exacerbation and quiescence of these lesions at an interval of about 3 months. The lesions on the tongue and right buccal mucosa often shrank to normal size, but increased to a size of 3 × 1 × 1 cm 3 , and he had episodes of bleeding from these lesions. The intraoral and cutaneous lesions had increased in size and number over the years. There was no family history of such lesions. Laboratory investigations showed normal RBC, WBC, and platelet count, Hb 9 g/dl, liver function tests within normal limits, normal bleeding time and clotting time, and normal blood urea nitrogen and creatinine levels. Endoscopy done 2 years back reported multiple bluish hemangiomas, ranging from 0.5 to 2 cm, without bleeding in the glottis and esophagus. He was not willing for biopsy. A clinical diagnosis of Bean syndrome was made based on the multiple mucocutaneous manifestations.
Figure 1: Clinical photograph showing lesion on the right buccal mucosa

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Figure 2: Clinical photograph showing lesions in the left lateral border of tongue

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Figure 3: Clinical photograph showing lesions on the dorsum of tongue

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Figure 4: Clinical photograph showing lesions in the left buccal mucosa

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Figure 5: Clinical photograph showing lesions in the posterior palate and ventral tongue

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Figure 6: Clinical photograph showing lesions in the left neck region

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Figure 7: Clinical photograph showing lesions in the right abdomen

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   Discussion Top


In 1860, Gascoyen first described the association between cutaneous and visceral hemangiomas and GI bleeding. [3] He reported a case of an anemic patient with multiple hemangiomas of the skin, GI tract (GIT), and parotid gland. In 1958, Bean reported a case with similar findings and pioneered the descriptive term blue rubber bleb nevus syndrome (BRBNS). The three different patterns of cutaneous lesions as described by Bean include:

  1. Multiple protuberant blue bloody sacs that are easily compressible;
  2. large hemangiomas that may lead to obstruction of the airway, alimentary canal, or other vital structures; and
  3. Pleomorphic flat blue lesions, occasionally with black stippling, that merge with adjacent normal skin. [1],[3],[4],[8]
Bean's description differentiated BRBNS from a heterogeneous group of vascular lesions. This rare systemic disorder affects both men and women of all ages and races. Most cases reported have sporadic occurrence; however, several families have demonstrated autosomal dominant inheritance patterns with significant penetrance. [4],[5],[6],[8]

The reported cases were mainly from the United States (20%), Japan (15%), Spain (9%), Germany (9%), China (6%), and France (6%), and much lesser cases have been reported from other countries, affecting whites more than blacks. [9] Medline search identified about 200 cases of BRBNS, and skin and mucosa, GIT, CNS, liver, and muscle were affected in the decreasing order, with minor distribution of other organs as well. [9] Hyperhidrosis is frequently seen with all three types of lesions described by Bean. Lesions have been documented throughout the entire body surface. A predisposition for the trunk and upper extremities is noticed. In addition to cutaneous involvement, vascular lesions of the GIT may also be present. Cavernous hemangiomas may be present anywhere from the oral mucosa to the anal mucosa, with the highest incidence occurring in the small bowel. [3],[4],[7] Unlike cutaneous lesions, mucosal hemangiomas of the GIT are friable and prone to bleeding, resulting in significant occult blood loss and subsequent anemia. [2],[3],[4],[7],[8],[10]

Although cavernous hemangiomas of the skin and GIT are the hallmarks of BRBNS, other multiple areas of involvement have been documented in literature. Hemangiomas have been reported in the brain, conjunctiva, iris, retina, oropharynx, nasopharynx, lung, pleura, heart, peritoneal cavity, mesentery, liver, penis, uterus, urinary bladder, spleen, joint capsule, and skeletal muscle. [1],[5],[7],[10] Orthopedic involvement with skeletal bowing of long bones and pathologic fractures, hypertrophy, and articular involvement have also been documented. [1],[7],[8] Histologic examination of the lesion reveals ectatic vascular channels lined with a single layer of flattened or cuboidal endothelial cells surrounded by a fibromuscular wall. This is not pathognomonic to the lesion. Occasionally, proliferation of the eccrine gland has been described, which accounts for the hyperhidrosis observed clinically. [3],[5],[6],[7],[8],[9] The diagnosis of BRBNS remains a clinical and histologic diagnosis. The discovery of cutaneous hemangiomas indicates further testing for anemia, occult blood loss, and visceral involvement. Magnetic resonance imaging and other imaging techniques have proved pivotal in describing the extent of visceral involvement. Recently, the role of labeling red blood cells with technetium Tc-99m to quantify visceral involvement has been described. [10] The stasis produced by the dilated venous malformation promotes focal accumulation of the labeled red blood cells in the skin and GIT. Magnetic resonance angiography has recently shown promising results in identifying vascular abnormalities. These imaging techniques may prove to be efficacious in determining visceral involvement in BRBNS. [10]

The lesions in Bean syndrome are really vascular malformations with regard to their histologic characteristics. [3],[4],[9] Hemangiomas have hyperplastic endothelium, while the vascular malformations are coated by normal endothelium. In children, hemangiomas tend to grow considerably during the first 6 months of life, moderately in the second semester, and decrease in size from 12 months of age on, and 95% of them will have disappeared by the time the child reaches 10 years of age. Vascular malformations do not usually present this behavior. [2],[3],[4],[10] In the BRBNS, intestinal lesions are more frequent in the small intestine, although any anatomical site, from the mouth to the anus, may be affected. When the colon is affected, they are more frequent in the rectal or distal areas. [3],[4],[7] Venous malformations in the sublingual region coinciding with similar lesions in the central nervous system have been reported. The clinical presentation varies in appearance, number, size, and distribution of the lesions. [2],[3],[5],[8],[10]

The nevi are often present in early childhood, and they may grow, increase in number, and lead to complications. [3],[4],[7],[10] Some patients report painful lesions, which may be due to the contraction of surrounding smooth muscles. [3],[4],[8],[10] There are also descriptions of lesions that cause increased sweating, possibly due to the proximity of the nevi to sweat glands. [4],[7] Thrombosis and calcification, as well as consumptive coagulopathy and thrombocytopenia may occur within the nevi. [3],[4],[6],[7] This syndrome has predominantly autosomal transmission. [1],[4],[6],[7]

The treatment may be by surgery, by local or systemic corticosteroid therapy, or by laser photocoagulation. [1],[6],[9],[10] Since in our case there were no cosmetic concern, the patient did not want to get the lesions excised. The intestinal nevi, in contrast to the cutaneous nevi, are easily injured and can bleed profusely. The bleeding may be massive, requiring transfusion or surgery. The bleeding may also be occult, and when it is chronic, it may lead to iron-deficiency anemia. [1],[3],[4],[6],[7] Our patient never presented any bleeding. Malignant transformation of these hemangiomas has not yet been reported.

BRBNS must be differentiated from hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome), Maffucci syndrome, Klippel-Trenaunay-Weber syndrome, Sturge-Weber syndrome, multiple glomus tumor, and Kaposi sarcoma. In inherited hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome), patients may present palindromic epistaxis during early life and telangiectasias during adolescence. In this condition, the GI lesions are similar, but the skin lesions are morphologically and histologically different. They are punctiform, red-bluish, and never reach the dimensions of those of the BRBNS. They affect the mucosa, lips, face, trunk, lungs, intestines, fingers, and lower limbs, but the sublingual lesions are very suggestive. [1],[3],[4],[6],[9],[10] The Klippel-Trenaunay syndrome presents varicosities, hypertrophia, soft tissue and bone deformities, arteriovenous fistulas, as well as "port wine nevus," generally located in just one of the extremities. [5],[6],[10] The Maffucci syndrome characterized by diffuse vascular malformations in the skin and soft tissues, associated with bone malformations and chondrodysplasias is another condition to be considered in differential diagnosis, but GIT lesions do not occur. [1],[3],[4],[6],[8],[10] There are other conditions in which vascular skin lesions are associated with lesions in other organs, such as meninges in the Sturge-Weber syndrome, brain and retina in the von Hippel-Lindau syndrome, and spinal cord in the Cobb syndrome and others. [6],[8],[10]


   Conclusion Top


In this paper, we have highlighted a few clinical presentations, investigations, and treatment options of blue bleb syndrome. Though rare, it can present in variable clinical severity. Clinicians should be aware of such myriad of presentation of Bean syndrome. Excessive bleeding may be the common complication expected.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Walshe MM, Evans CD, Warin RP. Blue rubber bleb naevus. Br Med J 1966;2:931-2.  Back to cited text no. 1
    
2.
Dobru D, Seuchea N, Dorin M, Careianu V. Blue rubber bleb syndrome: Case report and literature review. Rom J Gastroenterol 2004;13:237-40.  Back to cited text no. 2
    
3.
Camilleri M, Chadwick VS, Hodgson HJ. Vascular anomalies of the gastrointestinal tract. Hepatogastroenterology 1984;31: 149-53.  Back to cited text no. 3
    
4.
Liu Q, Chen YP, Li YM. Blue rubber bleb nevus syndrome: A report of one case associated with recurrent epistaxis. Chin Med J (Engl) 2007;120:731-3.  Back to cited text no. 4
    
5.
Moodley M, Ramdial P. Blue rubber bleb nevus syndrome: Case report and review of the literature. Pediatrics 1993;92:160-2.  Back to cited text no. 5
    
6.
Xu Y, Zhou B, Zhang M, Luo D. An unusual case of blue rubber bleb nevus syndrome with unilateral linear distribution. Indian J Dermatol Venereol Leprol 2013;79:269-70.  Back to cited text no. 6
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7.
Öksüzoglu BC, Öksüzoglu G, Çakir U, Bayir T, Esen M. Blue rubber bleb nevus syndrome. Am J Gastroenterol 1996; 91:780-2.  Back to cited text no. 7
    
8.
McKinlay JR, Kaiser J, Barrett TL. Blue rubber bleb nevus syndrome. Cutis 1998;62:97-8.  Back to cited text no. 8
    
9.
Jin XL, Wang ZH, Xiao XB, Huang LS, Zhao XY. Blue rubber bleb nevus syndrome: A case report and literature review. World J Gastroenterol 2014;20:17254-9.  Back to cited text no. 9
    
10.
Wirf FA, Lowitt MH. Diagnosis and treatment of cutaneous vascular lesions. Am Fam Physician 1998;57:765-73.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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