Blue Rubber Bleb Nevus Syndrome: Immunohistochemical Study

Abstract

Blue rubber bleb nevus is a rare entity consisting of distinctive angiomas in the skin and gastrointestinal tract, leading to occult or profound gastrointestinal bleeding and chronic anemia. The efficacy has been documented of systemic treatment with corticoids, interferon, vincristine, and, more recently, subcutaneous octreotide in the presence of active lesion proliferation or disseminated intravascular coagulation. A case of blue rubber bleb nevus syndrome with oral hemangiomas in a 24-year-old woman is reported. The surgical specimens were subjected to immunohistochemical study, which showed all of the hemangiomas to be in an inactive phase.

Blue rubber bleb nevus syndrome: novel lymphangiomatosis-like growth pattern within the uterus and immunohistochemical analysis

“…Lesional endothelial cells in the uterus immunohistochemically expressed the endothelial-related anitgens CD34, CD31, and factor VIII-related antigen. There has been only one previous immunohistochemical study of BRBNS, which analyzed cutaneous vascular lesions and showed results similar to ours with strong CD31, focal factor VIII related antigen, and negative Ki-67 expression [8]. Our data provide support for lymphatic differentiation in the vascular proliferation of BRBNS via focal expression of D2-40, a marker considered specific for lymphatic endothelium, and absence of a continuous subendothelial basement membrane, as evidenced by patchy collagen type IV immunoexpression [5,[9][10][11].…”

Elevated expression of c-kit in small venous malformations of blue rubber bleb nevus syndrome

“…4,7–8 Our results together with published data suggest that this moderate but steady increase of vessel size is based on the low number of dividing endothelial cells. 9 …”