RT Journal
A1 Steinert, Andre F.
A1 Reppenhagen, Stefan
A1 Baumann, Bernd
A1 Rudert, Maximilian
A1 Nöth, Ulrich
T1 Total Knee Arthroplasty in a Patient with Erdheim-Chester Disease with Massive Joint DestructionA Case Report
JF The Journal of Bone & Joint Surgery
JO The Journal of Bone & Joint Surgery
YR 2011
FD April 6
VO 93
IS 7
SP e29 1
OP 4
DO 10.2106/JBJS.J.00025
UL http://dx.doi.org/10.2106/JBJS.J.00025
AB 
Erdheim-Chester disease was named after William Chester, who described the disease in 1930, and the Viennese pathologist Jakob Erdheim. Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis that causes a systemic disease with involvement of the tubular long bones, sparing the axial skeleton, as well as the hands and feet1. The prognosis depends in large part on the extent and distribution of extraosseous manifestations, which most commonly includes diabetes insipidus; these manifestations can also include involvement of the retroperitoneum, skin, lung, and heart and include painless bilateral exophthalmus in >50% of cases2-4. Together with bone pain that arises from bone destruction affecting nociceptors within periosteum and bone marrow, mainly of the lower limbs, these clinical symptoms lead to the diagnosis of Erdheim-Chester disease. The diagnosis is usually based on typical radiographic findings, with symmetric bilateral osteosclerosis involving metaphyseal and diaphyseal regions of the long bones with sparing of the epiphyses. Bone scintigraphy reveals increased tracer uptake in Erdheim-Chester disease lesions, while magnetic resonance imaging shows replacement of the normal fatty bone marrow as well as periostitis2-6.