LymphangiomatosisD18.1

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Multifocal lymphangioendotheliomatosis with thrombocytopenia

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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.

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.

Maffucci syndrome; infant hemangioma; blue-rubber bleb nevus syndrome; hemangiodothelioma, type Dabska

Complication(s)This section has been translated automatically.

thrombocytopenia, GI bleeding

LiteratureThis section has been translated automatically.

  1. North PE et al (2004) Multifocal lymphangioendotheliomatosis with thrombocytopenia. Arch Dermatol 140: 599-606

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer