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Multifocal Congenital Lymphangioendotheliomatosis Without Gastrointestinal Bleeding and/or Thrombocytopenia

Khamaysi, Ziad MD*; Bergman, Reuven MD†‡

The American Journal of Dermatopathology: December 2010 - Volume 32 - Issue 8 - p 804-808
doi: 10.1097/DAD.0b013e3181dfd100
Original Study

A 5-month-old male infant presented with an increasing number of widespread asymptomatic violaceous cutaneous macules, papules, and nodules since birth. He is 1 of the 2 identical twins born to unrelated healthy parents. Histology revealed proliferation of dilated thin-walled vascular channels lined by bland endothelial cells in the dermis and subcutis. In some of the vascular channels, there were formations of intravascular papillae surrounded by hobnail endothelial cells. Immunohistochemistry demonstrated positive staining for CD31, CD34 factor VIII and vascular endothelial growth factor-3 (VEGFR-3) and negative staining for D2-40 and latency-associated nuclear antigen-1 (LANA-1). The clinical and histologic findings were compatible with multifocal congenital lymphangioendothelionmatosis with thrombocytopenia, except that a year of follow-up was uneventful and no gastrointestinal bleeding or thrombocytopenia was recorded.

From the *Department of Dermatology; and †Dermatopathology Service, Rambam Health Care Campus, and the Bruce Rappaport Faculty of Medicine, Technion, Israel Institute of Technology, Haifa, Israel.

Reprints: Prof. Reuven Bergman, MD, Department of Dermatology, Rambam Medical Center, Haifa, Israel (e-mail: r_bergman@rambam.health.gov.il).

© 2010 Lippincott Williams & Wilkins, Inc.