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Table 1 Diagnostic criteria of ECD

From: Erdheim-Chester Disease: a comprehensive review of the literature

Radiology Radiography Bilateral symmetric diametaphyseal osteosclerosis of long bones
99mTc Bone Scintigraphy Symmetric and abnormally strong 99mTc labeling of the distal ends of the long bones
Histology Microscopic Environment Non Langerhans histiocytes with foamy or eosinophillic cytoplasm, polymorphic granulomae and fibrosis, xanthogranulomatosis, proliferating fibroblasts, lymphocytic aggregates, Touton giant cells
Histiocyte Immunostaining CD68(+), CD1a(−), S-100(negative/low) *
Histiocyte Ultrastructure Lack of Birbeck granules
  1. (*) Pathological confirmation of CD68(+), CD1a(−) histiocytes is both sufficient and mandatory for the diagnosis of ECD.