An unusual case of Erdheim-Chester disease with features of Langerhans cell histiocytosis.
Erdheim-Chester disease (ECD) and Langerhans cell histiocytosis (LCH) are both exceedingly rare histiocytic proliferations that can involve the skeletal system. We report on a case of ECD with some features suggestive of LCH. Radiographs demonstrated a large lytic lesion in the left femur, with multiple lesions of sclerosis involving both distal femurs and tibias. Both the lytic lesion and a sclerotic lesion were biopsied and demonstrated distinctive histologic features characteristic of ECD in the tibia and features of LCH in the femur. The clinical/radiologic and pathologic features that distinguish ECD and LCH as distinct entities are reviewed, and the underlying biological connection between them is discussed.
Furmanczyk
Paul S
PS
Department of Pathology, University of Washington Medical Center, 1959 NE Pacific, P.O. Box 356100, Seattle, WA 98195-6100, USA. pfurman@u.washington.edu
Bruckner
James D
JD
Gillespy
Thurman
T
Rubin
Brian P
BP
D031249:Erdheim-Chester Disease / Q000150:complications / Q000473:pathology*; D005260:Female; D005269:Femur / Q000473:pathology; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008875:Middle Aged; D010146:Pain / Q000209:etiology; D035583:Rare Diseases; D013977:Tibia / Q000473:pathology