Abstract
Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis with classic radiographic findings of patchy or diffuse osteosclerosis predominantly involving the long bones in a bilaterally symmetrical pattern. A 49-year-old woman presented with diffuse lymphadenopathy, painful skin lesions, and constitutional symptoms. Recent history was significant for a nontraumatic fracture of the tibia 3 weeks prior to admission. Physical examination and laboratory studies were notable for lower extremity pain and swelling, nodular lesions on the skin, and normocytic, normochromic anemia. Plain radiographs showed a lytic pattern of destruction with a superimposed fracture in the left proximal tibia. MRI showed focal bone marrow replacement extending from the subchondral bone to the tibial diaphysis. Excisional lymph node and skin biopsies of the lesions demonstrated a CD-68 positive, S-100 variable, and CD1a-negative histiocytic cell proliferation filling the dermis and completely replacing the sampled lymph node with an accompanying chronic inflammatory infiltrate and fibrosis, pathognomonic for ECD. We report an unusual case of ECD presenting initially as diffuse, painful lymphadenopathy, and subsequently demonstrating a lytic lesion of the tibia underlying a nontraumatic fracture.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 835-840 |
| Number of pages | 6 |
| Journal | Skeletal Radiology |
| Volume | 43 |
| Issue number | 6 |
| DOIs | |
| State | Published - 2014 |
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Keywords
- Erdheim-Chester disease
- Histiocytosis
ASJC Scopus subject areas
- Radiology Nuclear Medicine and imaging
- Medicine(all)
Cite this
Erdheim-Chester disease : An unusual presentation of an uncommon disease. / Bindra, Jasjeet; Lam, Alexander; Lamba, Ramit; VanNess, Michael; Boutin, Robert D.
In: Skeletal Radiology, Vol. 43, No. 6, 2014, p. 835-840.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Erdheim-Chester disease
T2 - An unusual presentation of an uncommon disease
AU - Bindra, Jasjeet
AU - Lam, Alexander
AU - Lamba, Ramit
AU - VanNess, Michael
AU - Boutin, Robert D
PY - 2014
Y1 - 2014
N2 - Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis with classic radiographic findings of patchy or diffuse osteosclerosis predominantly involving the long bones in a bilaterally symmetrical pattern. A 49-year-old woman presented with diffuse lymphadenopathy, painful skin lesions, and constitutional symptoms. Recent history was significant for a nontraumatic fracture of the tibia 3 weeks prior to admission. Physical examination and laboratory studies were notable for lower extremity pain and swelling, nodular lesions on the skin, and normocytic, normochromic anemia. Plain radiographs showed a lytic pattern of destruction with a superimposed fracture in the left proximal tibia. MRI showed focal bone marrow replacement extending from the subchondral bone to the tibial diaphysis. Excisional lymph node and skin biopsies of the lesions demonstrated a CD-68 positive, S-100 variable, and CD1a-negative histiocytic cell proliferation filling the dermis and completely replacing the sampled lymph node with an accompanying chronic inflammatory infiltrate and fibrosis, pathognomonic for ECD. We report an unusual case of ECD presenting initially as diffuse, painful lymphadenopathy, and subsequently demonstrating a lytic lesion of the tibia underlying a nontraumatic fracture.
AB - Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis with classic radiographic findings of patchy or diffuse osteosclerosis predominantly involving the long bones in a bilaterally symmetrical pattern. A 49-year-old woman presented with diffuse lymphadenopathy, painful skin lesions, and constitutional symptoms. Recent history was significant for a nontraumatic fracture of the tibia 3 weeks prior to admission. Physical examination and laboratory studies were notable for lower extremity pain and swelling, nodular lesions on the skin, and normocytic, normochromic anemia. Plain radiographs showed a lytic pattern of destruction with a superimposed fracture in the left proximal tibia. MRI showed focal bone marrow replacement extending from the subchondral bone to the tibial diaphysis. Excisional lymph node and skin biopsies of the lesions demonstrated a CD-68 positive, S-100 variable, and CD1a-negative histiocytic cell proliferation filling the dermis and completely replacing the sampled lymph node with an accompanying chronic inflammatory infiltrate and fibrosis, pathognomonic for ECD. We report an unusual case of ECD presenting initially as diffuse, painful lymphadenopathy, and subsequently demonstrating a lytic lesion of the tibia underlying a nontraumatic fracture.
KW - Erdheim-Chester disease
KW - Histiocytosis
UR - http://www.scopus.com/inward/record.url?scp=84899499385&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84899499385&partnerID=8YFLogxK
U2 - 10.1007/s00256-013-1793-2
DO - 10.1007/s00256-013-1793-2
M3 - Article
C2 - 24366632
AN - SCOPUS:84899499385
VL - 43
SP - 835
EP - 840
JO - Skeletal Radiology
JF - Skeletal Radiology
SN - 0364-2348
IS - 6
ER -