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

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 involvi...

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Detalles Bibliográficos
Autores principales: Merai, Hema, Collas, David, Bhagat, Ashish, Mandalia, Uday
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Scientific Scholar 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7332465/
https://www.ncbi.nlm.nih.gov/pubmed/32637228
http://dx.doi.org/10.25259/JCIS_68_2020
Descripción
Sumario: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.