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osteitis fibrosa cystica; von Recklinghausen's disease of bone
Etiology:
- sustained secondary hyperparathyroidism
- most commonly seen in patients with chronic renal failure
- rarely seen with severe primary hyperparathyroidism [1]
Epidemiology:
1) now uncommon
2) in the past 25% of patients with hyperparathyroidism
Pathology:
1) increased osteoclastic resorption of calcified bone with replacement by soft tissue
- increased multinucleated osteoclasts
2) reduction in the number or trabecula
3) bone cysts
Radiology:
1) punched-out lesions in the skull
2) subperiosteal absorption of bone in the digits
3) humerus shows bone cysts & radiolucent lytic areas seen in long bones caused by brown tumors (collection of osteoclasts & fibrous material that appears brown secondary to hemosiderin deposition) [1]
4) subperiosteal resorption of bone, most prominently in phalanges of the hands [1]
* images [4]
Differential diagnosis:
- osteomalacia
- adynamic bone disease
General
osteitis
hyperparathyroidism
References
- Medical Knowledge Self Assessment Program (MKSAP) 11, 18.
American College of Physicians, Philadelphia 1998, 2018
- Medical Knowledge Self Assessment Program (MKSAP) 20
American College of Physicians, Philadelphia 2025
- Stedman's Medical Dictionary 26th ed, Williams &
Wilkins, Baltimore, 1995
- Harrison's Principles of Internal Medicine, 13th ed.
Isselbacher et al (eds), McGraw-Hill Inc. NY,
1994, pg 1253
- Ramon A, Berthod PE
Images in Clinical Medicine. Osteitis Fibrosa Cystica.
N Engl J Med 2020; 382:e15. March 12.
PMID: 32160666
https://www.nejm.org/doi/full/10.1056/NEJMicm1907828?query=TOC