Benign lymphoangioendothelioma(BL),also known as acquired progressive lymphangiomatosis,is a rare benign lymphoproliferative disease.At present,more than 70 cases have been reported worldwide.The clinical manifestations of this disease are usually slowly enlarging,large diameter of red or purple patches,the maximum diameter of which can reach more than 20 cm.The diagnosis of the disease depends on pathological examination.The pathological changes are characteristic,manifested as luminal hyperplasia involving the whole dermis,the formation of a large number of slit-like thin-walled lumen in the dermis,and even the formation of collagen segmentation.The proliferative luminal wall is mainly composed of a single layer of endothelial cells without significant cellular atypia or mitotic figures.The proliferative endothelial cells express lymphatic markers,including D2-40,Prox1,etc.,and have a low proliferation index of Ki-67,which can be distinguished from other malignant vascular neoplasms.The pathogenesis of this disease is still unclear,and currently there is a lack of effective treatment.This paper systematically reviews the clinical and pathological features,differential diagnosis and treatment progress of BL.
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