Erdheim-Chester Disease : A Case Report and Review of the Literature

© 2020 Published by Scientific Scholar on behalf of Journal of Clinical Imaging Science..

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans' cell histiocytosis characterized by xanthogranulomatous infiltration of foamy histiocytes surrounded by fibrosis. ECD may be asymptomatic or present as a multi-systemic disease with life-threatening manifestations, most commonly involving the skeletal system. Immunohistochemical staining demonstrates cells that are CD68+, CD1a-, and S100- with an absence of Birbeck granules. We report a case of a 69-year old male patient who presented with neurological symptoms - eventually thought to be separate to his diagnosis of ECD. It represents the ability to diagnose ECD based just on radiological findings in an otherwise asymptomatic individual.

Medienart:

E-Artikel

Erscheinungsjahr:

2020

Erschienen:

2020

Enthalten in:

Zur Gesamtaufnahme - volume:10

Enthalten in:

Journal of clinical imaging science - 10(2020) vom: 15., Seite 37

Sprache:

Englisch

Beteiligte Personen:

Merai, Hema [VerfasserIn]
Collas, David [VerfasserIn]
Bhagat, Ashish [VerfasserIn]
Mandalia, Uday [VerfasserIn]

Links:

Volltext

Themen:

Case Reports
Coated aorta sign
Erdheim-Chester Disease
Hairy kidney sign
Histiocytosis

Anmerkungen:

Date Revised 28.09.2020

published: Electronic-eCollection

Citation Status PubMed-not-MEDLINE

doi:

10.25259/JCIS_68_2020

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM312145942