Idiopathic familial acroosteolysis: Histomorphometric study of bone and literature review of the Hajdu-Cheney syndrome

Authors

  • James Udell MD,

    Rheumatology Fellow
    1. University of Pennsylvania School of Medicine and the Veterans Administration Medical Center, Philadelphia, Pennsylvania
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  • H. Ralph Schumacher Jr. MD,

    Professor of Medicine, Corresponding author
    1. University of Pennsylvania School of Medicine and the Veterans Administration Medical Center, Philadelphia, Pennsylvania
    • Rheumatology-Immunology Center (151K), Veterans Administration Medical Center, University and Woodland Avenues, Philadelphia, PA 19104
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  • Frederick Kaplan MD,

    Assistant Professor of Orthiopedic Surgery
    1. University of Pennsylvania School of Medicine and the Veterans Administration Medical Center, Philadelphia, Pennsylvania
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  • Michael D. Fallon MD

    Assistant Professor
    1. University of Pennsylvania School of Medicine and the Veterans Administration Medical Center, Philadelphia, Pennsylvania
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Abstract

We describe a 27-year-old man with familial acroosteolysis involving 9 fingers. Bone biopsy of an affected digit showed osteolysis with no tetracycline deposition, rare osteoclasts, increased vascularity, and numerous mast cells. In contrast, the iliac crest bone showed active bone remodeling and normal double-tetracycline labeling. We believe mast cells deserve further study as possible factors in this form of localized osteolysis.

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