title: Acral hemosideric lymphatic malformation.
abstract: BACKGROUND
  Cutaneous lymphatic malformations represent a group of heterogeneous diseases caused by developmental defects of lymphatic system.
  
  
  OBJECTIVE
  The purpose of this study was to report the clinical, histopathological and immunohistochemical features of a distinctive lymphatic malformation.
  
  
  METHODS
  Twelve patients with similar clinical and histopathological features were included in this study. Immunohistochemical staining of CD31, D2-40, Prox1 and Wilms tumor 1 (WT-1) were performed on all lesions.
  
  
  RESULTS
  All cases were either congenital lesions or developed during the first 2 years of life. All presented as red to brown papules or nodules on acral sites. Histopathologically, the lesions consisted of a dermal proliferation of flat or slit-like vessels lined with a single layer of endothelial cells. Hemosiderin or extravascular red blood cells were present in all cases. The constituent vessels expressed CD31, D2-40 and Prox1 and lacked expression of WT-1.
  
  
  CONCLUSIONS
  On the basis of the clinical, histopathological and immunohistochemical findings, our cases represent a unique type of lymphatic malformation that we believe is distinct from previously reported vascular proliferations. We propose the name of acral hemosideric lymphatic malformation for this entity.
authors.affiliation: Department of Dermatology, Xijing Hospital, Fourth Military Medical University, Xian, China.
authors.forename: Lei
authors.initials: L
authors.lastname: Wang
authors.forename: Tianwen
authors.initials: T
authors.lastname: Gao
authors.forename: Gang
authors.initials: G
authors.lastname: Wang
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D005786:Gene Expression Regulation; D006485:Hemosiderin / Q000096:biosynthesis*; D018398:Homeodomain Proteins / Q000096:biosynthesis; D006801:Humans; D044148:Lymphatic Abnormalities / Q000378:metabolism* / Q000473:pathology*; D008297:Male; D019408:Platelet Endothelial Cell Adhesion Molecule-1 / Q000096:biosynthesis; D012868:Skin Abnormalities / Q000378:metabolism* / Q000473:pathology*; D025521:Tumor Suppressor Proteins / Q000096:biosynthesis; D025721:WT1 Proteins / Q000096:biosynthesis; D000098551:Prospero-Related Homeobox 1 Protein
chemical_list: D018398:Homeodomain Proteins; D019408:Platelet Endothelial Cell Adhesion Molecule-1; D025521:Tumor Suppressor Proteins; D025721:WT1 Proteins; C583751:WT1 protein, human; D000098551:Prospero-Related Homeobox 1 Protein; D006485:Hemosiderin