Multifocal lymphangioendotheliomatosis with thrombocytopenia

Multifocal lymphangioendotheliomatosis with thrombocytopenia is an extremely rare disease. This condition manifests as diffuse congenital vascular lesions in the skin and gastrointestinal tract leading to severe gastrointestinal bleeding and thrombocytopenia. Histopathologic and immunohistochemical...

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Bibliographic Details
Published in:Journal of the American Academy of Dermatology 2006-05, Vol.54 (5), p.S214-S217
Main Authors: Yeung, Jensen, Somers, Gino, Viero, Sandra, Pope, Elena
Format: Article
Language:English
Subjects:
Adrenal Cortex Hormones - therapeutic use
Biological and medical sciences
Dermatology
Gastrointestinal Tract - blood supply
Hemangioendothelioma - complications
Hemangioendothelioma - congenital
Hemangioendothelioma - drug therapy
Hemangioendothelioma - pathology
Hematemesis - etiology
Hematologic and hematopoietic diseases
Humans
Immunohistochemistry - methods
Infant, Newborn
Lymphangioma - complications
Lymphangioma - congenital
Lymphangioma - drug therapy
Lymphangioma - pathology
Male
Medical sciences
Platelet diseases and coagulopathies
Skin - blood supply
Skin - pathology
Staining and Labeling
Thrombocytopenia - etiology
Vincristine - therapeutic use
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Description
Summary:Multifocal lymphangioendotheliomatosis with thrombocytopenia is an extremely rare disease. This condition manifests as diffuse congenital vascular lesions in the skin and gastrointestinal tract leading to severe gastrointestinal bleeding and thrombocytopenia. Histopathologic and immunohistochemical studies of vascular lesions demonstrate a lymphatic endothelial cell origin. Treatment often is not satisfactory. We herein describe a 4-week-old infant with this uncommon clinicopathologic entity.
ISSN:0190-9622
1097-6787
DOI:10.1016/j.jaad.2005.07.029