Synonym(s)
Multifocal lymphangioendotheliomatosis with thrombocytopenia
DefinitionThis section has been translated automatically.
Congenital or acquired in early childhood angiodysplasia characterized by excessive ectasia of abnormal lymphatic vessels
ManifestationThis section has been translated automatically.
Already present at birth or acquired in early childhood.
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LocalizationThis section has been translated automatically.
Diffuse skin infestation (trunk and extremities), soft tissue, bones, gastrointestinal tract.
Clinical featuresThis section has been translated automatically.
Very variable image with nodules, cysts, spots as well as extensive, soft to soft-elastic swellings of the grey-blue or brown tinted soft tissue. S.a. Lymphangioma circumscriptum.
HistologyThis section has been translated automatically.
Holey penetration of the dermis, subcutaneous and subcutaneous fatty tissue through dilated and anastomosing thin-walled vessels reminiscent of Swiss cheese. Endothelia with irregular protrusion (hobnail endothelia). Also detection of papillary protrusions. Pronounced hemosiderin deposits and lymphocytic infiltrates. Extramedullary hematopoiesis possible.
Differential diagnosisThis section has been translated automatically.
Complication(s)This section has been translated automatically.
thrombocytopenia, GI bleeding
LiteratureThis section has been translated automatically.
- North PE et al (2004) Multifocal lymphangioendotheliomatosis with thrombocytopenia. Arch Dermatol 140: 599-606
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Outgoing links (5)
Blue rubber bleb nevus syndrome; Infant haemangioma (overview); Lymphangioma circumscriptum; Maffucci syndrome; Papillary intralymphatic angioendothelioma;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer