title: Expanding the phenotype of multifocal lymphangioendotheliomatosis with thrombocytopenia.
abstract: Multifocal lymphangioendotheliomatosis with thrombocytopenia is characterized by vascular skin and gastrointestinal (GI) tract lesions, thrombocytopenia, and GI bleeding. The first patient had scattered red macules and subcutaneous nodules on the skin with involvement of the lungs, liver, omentum, and right kidney. At 10 months of age he continues to have severe GI bleeding. The second patient had innumerable vascular plaques on the skin plus muscle, bone, lung, liver, and brain involvement. She died from respiratory failure at 8 months of age due to brainstem involvement. Both patients required aggressive management of GI bleeding, but had quite different skin findings and long-term outcomes.
authors.affiliation: Department of Dermatology, Medical College of Wisconsin, Milwaukee, Wisconsin 53226, USA. mmaronn@yahoo.com
authors.forename: Mandi
authors.initials: M
authors.lastname: Maronn
authors.forename: Kristina
authors.initials: K
authors.lastname: Catrine
authors.forename: Paula
authors.initials: P
authors.lastname: North
authors.forename: Meghen B
authors.initials: MB
authors.lastname: Browning
authors.forename: Joseph E
authors.initials: JE
authors.lastname: Kerschner
authors.forename: Richard
authors.initials: R
authors.lastname: Noel
authors.forename: Beth A
authors.initials: BA
authors.lastname: Drolet
authors.forename: Michael
authors.initials: M
authors.lastname: Kelly
mesh_terms: D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000209:etiology*; D041981:Gastrointestinal Tract / Q000098:blood supply / Q000473:pathology; D006801:Humans; D007223:Infant; D008168:Lung / Q000473:pathology; D008297:Male; D010641:Phenotype; D012867:Skin / Q000098:blood supply / Q000473:pathology; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000473:pathology* / Q000503:physiopathology*; D013921:Thrombocytopenia / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology*; D054079:Vascular Malformations / Q000150:complications / Q000473:pathology / Q000503:physiopathology