title: The First Reported Case of Erdheim-Chester Disease in Egypt with Bilateral Exophthalmos, Loss of Vision, and Multi-Organ Involvement in a Young Woman.
abstract: BACKGROUND
  Erdheim-Chester disease is a rare non-Langerhans-cell histiocytosis of unknown etiology with multi-organ involvement.
  
  
  METHODS
  A 19-year-old woman presented with orthopnea, severe fatigue, bilateral exophthalmos, and gradual loss of vision. She had anemia and mild leucocytosis related to chronic illness. Marked left side pleural effusion and massive pericardial effusion with bilateral hydronephrosis were detected by plain X-ray, echocardiography, and computed tomography, respectively. Retro-orbital tissue and bone marrow biopsy revealed histiocytic infiltration, which was CD68-positive and CD1a-negative.
  
  
  CONCLUSIONS
  This report describes the first case presentation of Erdheim-Chester disease in our country. This case report may advance our understanding of an orphan disease. Our patient's young age and stable clinical status may allow long-term follow-up of treatment results.
authors.affiliation: Department of Internal Medicine, Zagazig University, Faculty of Medicine, Zagazig, Egypt.
authors.forename: Emad E
authors.initials: EE
authors.lastname: Abdellateef
authors.affiliation: Department of Internal Medicine, Zagazig University, Faculty of Medicine, Zagazig, Egypt.
authors.forename: Ayman R
authors.initials: AR
authors.lastname: Abdelhai
authors.affiliation: Department of Clinical Pathology, Zagazig University, Faculty of Medicine, Zagazig, Egypt.
authors.forename: Heba H
authors.initials: HH
authors.lastname: Gawish
authors.affiliation: Department of Radiology, Zagazig University, Faculty of Medicine, Zagazig, Egypt.
authors.forename: Ghada A
authors.initials: GA
authors.lastname: Abdulmonaem
authors.affiliation: Department of Pathology, Zagazig University, Faculty of Medicine, Zagazig, Egypt.
authors.forename: Eman H
authors.initials: EH
authors.lastname: Abdelbary
authors.affiliation: Department of Internal Medicine, Zagazig University, Faculty of Medicine, Zagazig, Egypt.
authors.forename: Ahmed I
authors.initials: AI
authors.lastname: Ahmed
mesh_terms: D001766:Blindness / Q000209:etiology; D004417:Dyspnea / Q000209:etiology; D004534:Egypt; D031249:Erdheim-Chester Disease / Q000150:complications* / Q000175:diagnosis*; D005094:Exophthalmos / Q000209:etiology; D005260:Female; D006801:Humans; D006869:Hydronephrosis / Q000209:etiology; D010490:Pericardial Effusion / Q000209:etiology; D010996:Pleural Effusion / Q000209:etiology; D035583:Rare Diseases / Q000150:complications* / Q000175:diagnosis*; D055815:Young Adult