Reactive angioendotheliomatosis in an infant

Am J Dermatopathol. 1999 Feb;21(1):42-5. doi: 10.1097/00000372-199902000-00010.

Abstract

Reactive cutaneous angioendotheliomatosis (RCA) is an uncommon benign disease characterized by intravascular proliferation of endothelial cells. The observation of RCA in infants is exceedingly rare. We describe a case of RCA in a 3-month-old infant. The lesions were characterized by six small purpuric papules (1-2 mm in diameter), distributed on the thighs and neck. The general condition of the patient was good, with no lymphadenopathy, systemic involvement, or fever. The histopathologic features of a papule were characterized by the presence of cohesive aggregates of large mononucleated cells protruding into the lumina of dilated vessels and filling some of them completely. Neither an inflammatory infiltrate nor a proliferation of pericytes were present around blood vessels. Intravascular proliferating cells demonstrated positive staining for Ulex europaeus agglutinin 1 (UEA-1) and for Factor VIII-RA and CD34 antigens. The course of the disease was unremarkable with persistence of the lesions for 8 months; no treatment was started.

Publication types

  • Case Reports

MeSH terms

  • Antigens, CD34 / analysis
  • Female
  • Humans
  • Immunohistochemistry
  • Infant
  • Lectins / analysis
  • Lymphoma, Non-Hodgkin / metabolism
  • Lymphoma, Non-Hodgkin / pathology*
  • Plant Lectins*
  • Skin / chemistry
  • Skin / pathology
  • Skin Neoplasms / metabolism
  • Skin Neoplasms / pathology*
  • von Willebrand Factor / analysis

Substances

  • Antigens, CD34
  • Lectins
  • Plant Lectins
  • Ulex europaeus lectins
  • von Willebrand Factor