MULTIFOCAL LYMPHANGIOENDOTHELIOMATOSIS WITH THROMBOCYTOPENIA
By Gary M. White, MD
Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) describes a congenital vascular condition with lymphatic differentiation that may lead to a consumptive coagulopathy.
- The prognosis of MLT is poor, with high mortality, estimated as high as 65%.
The skin shows hundreds of congenital red-brown skin plaques as large as a few centimeters. The gastrointestinal tract may be similarly affected and severe GI bleeding may occur. Significant thrombocytopenia is associated, presumably from the Kasabach Merritt Syndrome.
Corticosteroids do not seem to help. Two infants with MLT responded well to sirolimus [PD 2016;33;e235].
Arch Dermatol. 2004;140(5):599-606
JAAD July 2012 Volume 67, Issue 1, Pages e62–e64
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