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.

Clinical

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.

Treatment

Corticosteroids do not seem to help. Two infants with MLT responded well to sirolimus [PD 2016;33;e235].

References

Arch Dermatol. 2004;140(5):599-606

JAAD July 2012 Volume 67, Issue 1, Pages e62–e64

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