The First Reported Case of Erdheim-Chester Disease in Egypt with Bilateral Exophthalmos, Loss of Vision, and Multi-Organ Involvement in a Young Woman

Erdheim-Chester disease is a rare non-Langerhans-cell histiocytosis of unknown etiology with multi-organ involvement. 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. Mar...

Full description

Saved in:
Bibliographic Details
Published in:The American journal of case reports 2016-05, Vol.17, p.360-370
Main Authors: Abdellateef, Emad E, Abdelhai, Ayman R, Gawish, Heba H, Abdulmonaem, Ghada A, Abdelbary, Eman H, Ahmed, Ahmed I
Format: Article
Language:English
Subjects:
Blindness - etiology
Dyspnea - etiology
Egypt
Erdheim-Chester Disease - complications
Erdheim-Chester Disease - diagnosis
Exophthalmos - etiology
Female
Humans
Hydronephrosis - etiology
Pericardial Effusion - etiology
Pleural Effusion - etiology
Rare Diseases - complications
Rare Diseases - diagnosis
Young Adult
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Erdheim-Chester disease is a rare non-Langerhans-cell histiocytosis of unknown etiology with multi-organ involvement. 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. 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.
ISSN:1941-5923
1941-5923
DOI:10.12659/AJCR.897479