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.
Important additional findings include vascular lesions in the GI tract and possibly other organ systems.
Severe gastrointestinal bleeding resulting from these lesions is likely and requires repeated blood transfusions.
Instead, the diagnosis is made based on clinical evidence and supporting histopathology that demonstrates the presence of positive lymphatic markers, such as podoplanin (D2-40) or lymphatic vessel endothelial hyaluronan receptor 1 (LYVE-1), as well as negative glucose transporter 1 (GLUT-1).
In terms of medical therapy, corticosteroids, thalidomide, vincristine, propranolol, octreotide, aminocaproic acid, bevacizumab,[10] and/or sirolimus[11] are available.