Erdheim-Chester disease in a child presenting with multiple jaw lesions.
BACKGROUND
Erdheim-Chester disease is a rare histiocytic disease entity related to juvenile xanthogranuloma. It is a systemic condition, usually occurs in adult, characterized by infiltration of foamy histiocytes within the bone and soft tissues.


RESULTS
We report a case of 13-year-old female patient who first presented with multiple osteolytic lesions of the jaws followed by bilateral symmetrical bone lesions affecting the lower extremities, as well as brain and abdominal involvement. Histological findings of the jaw lesions showed lipid-storing CD68 (+), CD1a (-) histiocytes with Touton type giant cells.


CONCLUSIONS
To the best of our knowledge, this is the first case of Erdheim-Chester disease with jaw bone lesions occurring as initial presenting symptom.
Nagatsuka
Hitoshi
H
Department of Oral Pathology and Medicine, Graduate School of Medicine and Dentistry, Okayama University, Okayama, Japan.
Han
Phuu Pwint
PP
Taguchi
Koji
K
Tsujigiwa
Hidetsugu
H
Gunduz
Mehmet
M
Fukunaga
Joji
J
Sugahara
Toshio
T
Asaumi
Junichi
J
Nagai
Noriyuki
N
D000293:Adolescent; D015703:Antigens, CD / Q000032:analysis; D015214:Antigens, Differentiation, Myelomonocytic / Q000032:analysis; D001932:Brain Neoplasms / Q000175:diagnosis; D003937:Diagnosis, Differential; D031249:Erdheim-Chester Disease / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007571:Jaw Diseases / Q000175:diagnosis*; D013577:Syndrome; D000097382:CD68 Molecule
D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; C067980:CD68 antigen, human; D000097382:CD68 Molecule