Erdheim-Chester disease: description of eight cases.
Erdheim-Chester disease, although rare, has a wide range of manifestations. It is characterized by the xanthomatous infiltration of tissues by spumous histiocytes, surrounded by fibrosis. The symptoms can vary from bone pain, diabetes insipidus, exophthalmos, xanthelasmas, cardiovascular involvement, bilateral adrenal enlargement, renal impairment, testis infiltration, interstitial lung disease to retroperitoneal fibrosis with perirenal and/or ureteral obstruction. We present eight cases, four of them with only breast involvement and the others with bone, cardiovascular, central nervous system, and renal involvement. All showed infiltrates of histiocytes and fibrosis on microscopic evaluation and positive CD68 and negative CD1a on immunohistochemical stains.
Rheumatology Section, José María Cullen Hospital, Av. Freyre 2150, (3000), Santa Fe, Argentina. susanarove@yahoo.com.ar.
S
S
Roverano
Rheumatology Section, José María Cullen Hospital, Av. Freyre 2150, (3000), Santa Fe, Argentina.
J
J
Gallo
Rheumatology Section, José María Cullen Hospital, Av. Freyre 2150, (3000), Santa Fe, Argentina.
A
A
Ortiz
Department of Pathology, José María Cullen Hospital, Av. Freyre 2150, (3000), Santa Fe, Argentina.
N
N
Migliore
Department of Pathology, José María Cullen Hospital, Av. Freyre 2150, (3000), Santa Fe, Argentina.
Mónica
M
Eletti
Rheumatology Section, José María Cullen Hospital, Av. Freyre 2150, (3000), Santa Fe, Argentina.
S
S
Paira
D000005:Abdomen / Q000000981:diagnostic imaging*; D000328:Adult; D001118:Argentina; D031249:Erdheim-Chester Disease / Q000000981:diagnostic imaging* / Q000473:pathology*; D005260:Female; D005355:Fibrosis; D006801:Humans; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed
Erdheim-Chester; Non-Langerhans histiocytosis; Xanthomatous tissue infiltration