The Journal of Clinical Endocrinology & Metabolism Vol. 92, No. 5 1590-1591
Copyright © 2007 by The Endocrine Society
Ivory Fingers: Monostotic Paget’s Disease of the Phalanges
Edward Calif,
Eugene Vlodavsky and
Shalom Stahl
The Unit of Hand Surgery (E.C., S.S.) and Institute of Pathology (E.V.), Rambam Medical Center, Haifa 31096, Israel
Address all correspondence and requests for reprints to: Edward Calif, M.D., The Unit of Hand Surgery, Rambam Medical Center, P.O. Box 9602, Haifa 31096, Israel. E-mail: edikal{at}hotmail.com.
Two otherwise healthy women (aged 33 and 46 yr, respectively) presented with similar histories of chronic dull pain, aggravated at night, in their left index and left fifth fingers, respectively. On physical examination, the first patient had a tender swelling of the proximal phalanx, with mildly restricted finger motion. The second patient had diffuse swelling and tenderness of the middle phalanx with intact movements. In both cases, the serum alkaline phosphatase concentrations were normal. In both patients, x-rays revealed widening and homogenous sclerosis of the involved phalanges (Fig. 1
). Both patients’ Tc-99m MDP bone scans demonstrated a "ring sign" of monostotic phalangeal involvement (Fig. 2). Biopsies demonstrated that the bone in both patients had an "ivory" consistency. Histological examination revealed extension of osteoid surfaces, irregular and coarse trabeculae, and a disordered mosaic pattern. The first patient was treated with oral alendronate 10 mg/d, whereas the second benefited from acetaminophen, with rapid pain alleviation.
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FIG. 1. Ivory phalanges. A, Antero-posterior roentgenogram of the index finger of case 1. B, Lateral roentgenogram of the fifth finger of case 2, featuring phalangeal widening, cortical thickening, sclerosis, coarse trabeculae, amorphous appearance, and obliteration of cortical-medullary boundaries of the proximal and middle phalanges, respectively.
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FIG. 2. Ring sign: Tc-99m MDP scintigraphic image of the hands in case 1 demonstrating intensely increased radionuclide uptake in the proximal phalanx of the index. No other bones were affected at skeletal survey.
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Paget’s disease, which afflicts mainly middle-aged and elderly adults (1), is monostotic in 15–30% of patients (2, 3, 4); hand involvement has been said to be rare. The incidence of Paget’s disease of the hand may be greater than generally believed and should be a part of the differential diagnosis when evaluating local hand pain when there are suggestive radiological findings. Scintigraphy can help confirm the diagnosis. Bone biopsy is indicated to exclude malignancy in those with apparent monostotic hand disease and recent-onset symptoms.
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Footnotes
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Disclosure Statement: The authors have nothing to declare.
Received June 21, 2006.
Accepted February 26, 2007.
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References
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- Friedman AC, Orcutt J, Madewell JE 1982 Paget disease of the hand: radiographic spectrum. Am J Roentgenol 138:691–693[Abstract/Free Full Text]
- Meunier PJ, Salson C, Mathieu L, Chapuy MC, Delmas P, Alexandre C, Charhon S 1987 Skeletal distribution and biochemical parameters of Paget’s disease. Clin Orthop Relat Res 217:37–44[Medline]
- Delmas PD, Meunier PJ 1997 The management of Paget’s disease of bone. N Engl J Med 336:558–566[Free Full Text]
- Ankrom MA, Shapiro JR 1998 Paget’s disease of bone (osteitis deformans). J Am Geriatr Soc 46:1025–1033[Medline]
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