Sunday, November 05, 2006

Blue Rubber Bleb Nevus Syndrome

Blue Rubber Bleb Nevus Syndrome (Bean Syndrome)

General: Onset after birth; autosomal dominant.

Ocular: Subconjunctival hemangioma with overlying fibrosis; raised hemorrhagic lesion near macula suggestive of a small arteriovenous malformation.

Clinical: Vascular lesions; cutaneous lesions found anywhere on the body; profuse sweating may occur over the skin lesions with pain or tenderness on palpation; visceral lesions are common; tender bluish papules on the trunk and extremities; colonic hemangiomas.

Bean WB. Blue rubber bleb naevi of the skin and gastrointestinal tract in vascular spiders and related lesions of the skin. Springfield, IL: Charles C. Thomas, 1958:178-185.

Chang EL, Rubin PA: Bilateral multifocal hemangiomas of the orbit in the blue rubber bleb nevus syndrome. Ophthalmology 2002; 109: 537-541.

Crompton JL, Taylor D. Ocular lesion in the blue rubber naevus syndrome. Br J Ophthalmol 1981; 65:133-137.

Moodley M, Ramdial P. Blue rubber bleb nevus syndrome: case report and review of the literature. Pediatrics 1993; 92:160-162.

Paules S, et al. Tender bluish papules on the trunk and extremities. Blue rubber-bleb nevus syndrome. Arch Dermatol 1993; 129:1505-1506.

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