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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. [2]: 596
Symptoms may not raise concern, or even be noted, until the disease process has advanced to a point where it causes restrictive compression of vital structures. Further, the occurrence of chylous effusions seems to be unrelated to the pathologic "burden" of the disease, the extent of involvement in any particular tissue or organ, or the age of ...
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.
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.
The direct cause of lymphatic malformation is a blockage of the lymphatic system as a fetus develops, although symptoms may not become visible until after the baby is born. The cause remains unknown. Why the embryonic lymph sacs remain disconnected from the rest of the lymphatic system is also not known. [7]
Although the exact cause of microvenular hemangioma is uncertain, it has been found in certain instances during pregnancy or after changes in hormonal contraception. [5] In certain circumstances, imbalanced sex hormones could potentially be the cause. [6] Microvenular hemangioma has also been associated with immunosuppression. [7]
Proliferating Angioendotheliomatosis has historically been divided into two groups, (1) a reactive, involuting type and (2) a malignant, rapidly fatal type. [1]: 598 ...
Organ involvement can also cause more specific symptoms. Bone: The most-frequently seen symptom in both unifocal and multifocal disease is painful bone swelling. The skull is most frequently affected, followed by the long bones of the upper extremities and flat bones. Infiltration in hands and feet is unusual.