title: A singular case of congenital self-healing histiocytosis with skin, liver and atypical eye involvement.
abstract: OBJECTIVE
  To describe a rare case of congenital self-healing Langerhans cell histiocytosis (CSHLCH) presenting with atypical eye involvement.
  
  
  METHODS
  Case report.
  
  
  METHODS
  A female newborn presented with purpuric lesions over the trunk, limbs, and face. Liver ultrasonography revealed hypoechogenic lesions with blurred borders. Biomicroscopy showed right posterior synechiae with fibrinoid deposits on the lens. At 7 months she presented with right acute glaucoma.
  
  
  RESULTS
  Biomicroscopy showed the presence of inflammatory pseudo-membrane covering the anterior surface of the lens, iris, and iridocorneal angle. Ab externo trabeculotomy was performed; access to the anterior chamber with capsulorrhexis forceps permitted a peeling of the pseudo-membrane with normalization of the intraocular pressure. Histologic examination of the membrane revealed an inflammatory tissue with CD1a and S-100 positive histiocytic cells.
  
  
  CONCLUSIONS
  This is the first case of CSHLCH describing acute glaucoma secondary to a pseudo-inflammatory membrane with typical histiocytic cells, occluding the iridocorneal angle.
authors.affiliation: Department of Surgery, Ophthalmology Unit, Institute for Maternal and Child Health Burlo Garofolo, Via dell’Istria 65/1 Trieste, Italy. parentin@burlo.trieste.it
authors.forename: Fulvio
authors.initials: F
authors.lastname: Parentin
authors.forename: Giovanna
authors.initials: G
authors.lastname: Ventura
authors.forename: Serena
authors.initials: S
authors.lastname: Pastore
authors.forename: Valentina
authors.initials: V
authors.lastname: Kiren
authors.forename: Chiara
authors.initials: C
authors.lastname: Bibalo
authors.forename: Stefano
authors.initials: S
authors.lastname: Pensiero
authors.forename: Loredana
authors.initials: L
authors.lastname: Lepore
mesh_terms: D018949:Antigens, CD1 / Q000276:immunology; D003289:Convalescence; D005260:Female; D005901:Glaucoma / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000151:congenital* / Q000276:immunology; D006801:Humans; D007223:Infant; D007429:Intraocular Pressure / Q000276:immunology; D057129:Iridocorneal Endothelial Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D008107:Liver Diseases / Q000000981:diagnostic imaging / Q000209:etiology / Q000276:immunology; D009418:S100 Proteins / Q000276:immunology; D012871:Skin Diseases / Q000209:etiology / Q000276:immunology; D014130:Trabeculectomy; D014463:Ultrasonography
chemical_list: D018949:Antigens, CD1; C419393:CD1a antigen; D009418:S100 Proteins