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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 .
There are three distinct types of lymphangioma, each with their own symptoms. They are distinguished by the depth and the size of abnormal lymph vessels, but all involve a malformation of the lymphic system. Lymphangioma circumscriptum can be found on the skin's surface, and the other two types of lymphangiomas occur deeper under the skin.
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.
Microvenular hemangioma appears as slow-growing, frequently numerous lesions, erythematous, violaceous, or moderately pruriginous macules, papules, or nodules without any symptoms. [3] They usually appear on the forearm. There have also been reports of lesions on the soles of the feet, forehead, legs, and chest. [4]
Proliferating Angioendotheliomatosis has historically been divided into two groups, (1) a reactive, involuting type and (2) a malignant, rapidly fatal type. [1]: 598 ...
An infantile hemangioma, also called a strawberry angioma, on a child's arm. Angiomas usually appear at or near the surface of the skin anywhere on the body, and may be considered bothersome depending on their location.
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)