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Figure 1 is an anteroposterior radiograph of the left shoulder that demonstrates innumerable sclerotic foci throughout the shoulder. Figures 2 and 3 are coronal and sagittal T1 weighted magnetic resonance images of the left shoulder joint demonstrating multiple, punctuate, hypointense foci throughout the bony structures.


Originally described in the early 1900s by Alberg-Schonberg, osteopoikilosis, or spotted bone disease, is an uncommon, benign bone dysplasia. Though sporadic cases have been identified, current evidence suggests that this condition is inherited in an autosomal dominant fashion. Thought to develop in childhood and progress until the closure of the growth plates, osteopoikilosis is characteristically asymptomatic; however, approximately 15%-20% of patients who suffer from this condition experience mild articular pain and have a joint effusion. (1)

Pathophysiologically, this condition consists of multiple enostoses, or bone islands, which are scattered throughout the skeleton--especially in the epiphyses and metaphyses of long tubular bones. Histologically, these radiodensities are comprised of compact lamellar bone and are identical to solitary bone islands that are commonly identified on radiographs. These lesions show a predilection for the knee, shoulder, pelvis, carpal, and tarsal bones. Less commonly, they can be located in the skull, ribs, mandible, and vertebral bodies. (2) Typically, the long axis of the sclerotic focus runs in parallel with the long axis of the involved osseous structure.

Osteopoikilosis can be diagnosed using a plain radiograph through the identification of multiple, variably shaped and sized radio-opaque spots clustered around joints and scattered throughout the skeleton. These lesions are discrete and typically symmetrical in appearance. Characteristically, osteopoikilosis does not demonstrate aggressive features; specifically, there is no associated periosteal reaction, cortical disruption, extension into adjacent structures, or soft tissue edema surrounding the site of sclerosis. Magnetic resonance (MR) examination demonstrates multiple round, hypointense lesions. (3)

Osteopoikilosis does not demonstrate increased uptake of radiopharmaceutical material on a nuclear medicine bone scan. This characteristic, in combination with stability over time, differentiates it from osteoblastic bone metastases, the most important differential diagnosis. On a nuclear medicine bone scan, metastatic bone lesions manifest as a region of increased metabolic activity or hot spot. Other differential diagnoses include: melorheostosis, mastocytosis, and tuberous sclerosis. (4)

While the natural course of osteopoikilosis is completely benign and requires no treatment, it can be associated with other abnormalities. In 25% of cases, it is associated with pea-sized fibrous epidermal papules and together these findings are known as Buschke-Olledorf syndrome. (4)

Knowledge of osteopoikilosis as a rare, benign condition is critical for optimal patient care and avoiding misdiagnosis.


(1.) Borman P, Ozoran K, Aydog S, et al. Osteopoikilosis: report of a clinical case and review of the literature. Joint Bone Spine 2002;69:230-233.

(2.) Khot R, Sikarwar JS, Gupta RP, et al. Osteopoikilosis: a case report. Ind J Radiol Imag 2005;15:453-454.

(3.) Tander B, Ceng|z K, D|ren B, et al. A case of osteopoikilosis mimicking metastases on MRI study. Turk J Phys Med Rehab 2006;52:85-87.

(4.) McLennan M. Radiology rounds. Can Fam Physician 1999;45:2315-2320.

Kelsey Flynt, BA; Sean Johnston, MD; and Harold R. Neitzschman, MD (Section Editor)

Ms. Flynt is a fourth year medical student at the University of Missouri in Columbia, Missouri. Dr. Johnston is a musculoskeletal and body imaging fellow at Tulane University Health Sciences Center in New Orleans. Dr. Neitzschman is a professor of radiology and the chairman of the Department of Radiology at Tulane University Health Sciences Center in New Orleans. Donald Olivares is the digital imaging specialist and graphic designer for the Department of Radiology at Tulane University Health Sciences Center in New Orleans.
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Title Annotation:Radiology Case of the Month
Author:Flynt, Kelsey; Johnston, Sean; Neitzschman, Harold R.
Publication:The Journal of the Louisiana State Medical Society
Article Type:Report
Date:Nov 1, 2010
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