multifocal lymphangioendotheliomatosis
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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.
References
Yeung J, Somers G, Viero S, Pope E. Multifocal lymphangioendotheliomatosis with thrombocytopenia. J Am Acad Dermatol. 2006 May;54(5 Suppl):S214-7. PMID: 16631943
Piggott KD, Riedel PA, Baron HI. Multifocal lymphangioendotheliomatosis with thrombocytopenia: a rare cause of gastrointestinal bleeding in the newborn period. Pediatrics. 2006 Apr;117(4):e810-3. PMID: 16585291
North PE, Kahn T, Cordisco MR, Dadras SS, Detmar M, Frieden IJ. Multifocal lymphangioendotheliomatosis with thrombocytopenia: a newly recognized clinicopathological entity. Arch Dermatol. 2004 May;140(5):599-606. PMID: 15148106
Bakaeen G, Winkler S, Bakaeen L, Rehani LA, Katsetos CD. Congenital macroglossal angiodysplasia ("Lymphangioendotheliomatosis"). Arch Pathol Lab Med. 2000 Sep;124(9):1349-51. PMID: 10975936
Loeffler JR, Duray PH, Sasaki CT. Macroglossal lymphangioendotheliomatosis. Report of a case in a pregnant woman. Arch Otolaryngol. 1984 Sep;110(9):600-3. PMID: 6477279