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Multifocal lymphangioendotheliomatosis, also known as congenital cutaneovisceral angiomatosis with thrombocytopenia and multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT), [1] is a skin condition that presents at birth with hundreds of red-brown plaques as large as several centimeters.
Lymphangiomatosis is a condition where a lymphangioma is not present in a single localised mass, but in a widespread or multifocal manner. It is a rare type of tumor which results from an abnormal development of the lymphatic system. [1]
Proliferating Angioendotheliomatosis has historically been divided into two groups, (1) a reactive, involuting type and (2) a malignant, rapidly fatal type. [1]: 598 ...
In the past, the diagnosis of "diffuse neonatal hemangiomatosis" included both infantile hemangioma (IH) and multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT). [8] With advances in medicine and vascular anomalies, there is now a differentiation between the diagnosis of IH, which is benign, and MLT, which has a high mortality rate.
Lymphatic malformations are benign slow-flow type of vascular malformation of the lymphatic system characterized by lymphatic vessels which do not connect to the normal lymphatic circulation.
Multifocal lymphangioendotheliomatosis (congenital cutaneovisceral angiomatosis with thrombocytopenia, multifocal lymphangioendotheliomatosis with thrombocytopenia) Multinucleate cell angiohistocytoma; Multiple cutaneous and uterine leiomyomatosis syndrome (leiomyomatosis cutis et uteri, multiple leiomyomatosis, Reed's syndrome)
Multiple distinct red globules are seen under a microscope, and a finely pigmented network is seen around the periphery. [8] Histologically, microvenular hemangioma is made up of venules with thin walls that branch erratically and have barely noticeable vascular lumina.
The cause of blue rubber bleb nevus syndrome is currently unknown. The syndrome is considered sporadic.A patient who is diagnosed with BRBNS likely has a family history of other multifocal venous malformations [8] which are a symptom of the disease.