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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.