Osteopoikilosis: report of a clinical case and review of the literature

Borman, Pınar; Özoran, Kürşat; Aydoǧ, Şükrü; Coskun, Selcuk

doi:10.1016/s1297-319x(02)00375-5

Cited by 43 publications

(30 citation statements)

References 18 publications

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“…Difficulty occurs when patients present for other reasons, such as joint pain, as described in a number of case reports. [10][11][12] In these situations, a review of previous imaging is essential to show that the lesion has remained stable over time. Correlation with a radionuclide technetium-99m bone scan can be performed when previous images are not available.…”

Section: Additional Testingmentioning

confidence: 99%

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Benign spotted bones: a diagnostic dilemma

Primio¹

2011

Canadian Medical Association Journal

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A young woman presented to the emergency department with acute knee pain after a minor fall. Radiographs showed no evidence of fracture or joint effusion, but scattered periarticular sclerotic foci of variable size were noted in the femur and tibia (Figure 1). These lesions were re cognized by the radiologist as typical for osteopoikilosis.S potted bones associated with benign conditions can have a very typical radiographic appearance and distribution; however, ambiguity in this appearance or a history of malignant disease can lead to diagnostic uncertainty and the need for further investigation. This clinical primer will focus on one characteristic benign condition, osteopoikilosis, its relevant differential diagnosis and workup.Osteopoikilosis was first described in 1915 by Albers-Schönberg as a sclerosing bone dysplasia of unknown cause.1 It is also referred to as spotted bones or osteopathia condensans disseminata. A diagnosis of exclusion, cases may be underreported. Prevalence in the general population is unknown, but an older retrospective review reported an estimated prevalence of 1 in 50 000. 2 The lesions have been described in all age groups, and although prevalence studies have shown a higher frequency among men, the apparently unequal sex distribution may be a result of referral bias in the literature (men are more likely than women to present to hospital with traumatic injuries requiring radiologic investigation). 2,3Osteopoikilosis exists in hereditary (autosomal dominant transmission) and sporadic forms [4][5][6][7] and is one of several bone dysplasias characterized by defective endochondral bone formation.5 Endochondral ossification refers to the formation of the long and flat bones, which begins from a primitive hyaline cartilaginous model. This process is in contrast to intramembranous ossification, which refers to direct transformation of condensed mesenchymal cells into cortical bone without a cartilaginous phase, as is typically seen in the formation of the skull bones. 5Most reported cases of osteopoikilosis are identified during the investigation of unrelated problems in which there is no clinical history suggestive of either malignant or systemic disease. 8,9 In such situations, no further workup is necessary. The characteristic radiologic feature is multiple, punctate, sclerotic, round or oval foci symmetrically distributed in a predominantly periarticular fashion within the epiphyseal and metaphyseal regions. 8,9 In the three patients described in this issue, most of the sclerotic foci are 1-2 mm, although some lesions measure up to 10 mm. Although further investigation is unnecessary in typical osteopoikilosis, when radionuclide bone scans are performed, their results are negative. In clinical and radiologic follow-up of osteopoikilosis, the lesions remain stable.Osteopoikilosis is typically an asymptomatic incidental finding, but it can be associated with other diseases. Most importantly, it must be differentiated from sclerotic metastases and other sclerosing dyplasias. • Osteopoik...

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Section: Additional Testingmentioning

confidence: 99%

“…[12][13][14][15][16][17] The uptake may be due to active remodeling of the bone, i.e., a process similar to the formation of calluses. 15 Although uptake of 99m Tc is unusual in osteopoikilosis and bone islands, it is commonly observed in connection with metastasis (Figure 3), sarcoidosis, mastocytosis and tuberous sclerosis.…”

Section: Additional Testingmentioning

confidence: 99%

Benign spotted bones: a diagnostic dilemma

Primio¹

2011

Canadian Medical Association Journal

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“…These findings (B) were compatible with osteopoikilosis. [1][2][3][4][5][6][7] There were similar patchy areas of sclerosis throughout both knees, the distal femurs, and the proximal tibias. A whole-body bone scan revealed intense, symmetrically increased uptake in the bilateral proximal and distal ulnas, distal radii, second and third proximal phalanges, bilateral distal tibias and fibulas, both calcanei, both patellas, and both tarsal bones.…”

Section: Figurementioning

confidence: 99%

Abnormal Bone Scan in an Adult With Osteopoikilosis

Yoon

Lee

et al. 2004

Clinical Nuclear Medicine

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Osteopoikilosis is rare, autosomal dominant bone disorder diagnosed by radiologic features. The findings include multiple small, circumscribed round areas of increased bone density distributed symmetrically in a periarticular location. The lesions cluster at the ends of long bones, around the acetabulum, and in the small bones of the hands and feet. Normal Tc-99m MDP uptake in osteopoikilosis on a bone scan may be used as a marker in distinguishing it from osteoblastic bone metastases, but there are a few previous reports of abnormal bone scan findings in osteopoikilosis among young patients. The authors describe a 40-year-old woman with osteopoikilosis characterized by classic radiologic findings and an abnormal bone scan.

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“…Osteopoikilosis (OPK) -a skeletal dysplasia described by Albers-Schönberg in 1915 -is characterised by the formation of densities in cancellous bone tissue, visible on imaging studies as speckles or streaky shadows located Wstęp Osteopoikilia (OPK) -opisana przez Albers-Schönber-ga w 1915 r. dysplazja szkieletu -polega na powstawaniu w tkance kostnej gąbczastej zagęszczeń widocznych w badaniach obrazowych w postaci cieni plamistych primarily in the area of the joints [1][2][3]. The condition is genetic in nature, showing autosomal dominant inheritance, with prevalence in the general population estimated at 1 in 50 000 [1].…”

Section: Introductionmentioning

confidence: 99%

“…The condition is genetic in nature, showing autosomal dominant inheritance, with prevalence in the general population estimated at 1 in 50 000 [1]. The majority of OPK cases are asymptomatic, however 15-20% of patients experience pain or joint inflammation [3]. Bone lesions are generally identified incidentally when imaging studies are performed for unrelated indications, usually post-trauma.…”

Section: Introductionmentioning

confidence: 99%

Osteopoikilosis associated with keloids formation, rheumatoid arthritis, myasthenia, Graves` disease and megaloblastic anaemia

Zimmermann-Górska¹

2014

Reumatologia

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Słowa kluczowe: osteopoikilia, bliznowce, towarzyszące choroby autoimmunologiczne. S t r e s z c z e n i eOsteopoikilia jest rzadką, uwarunkowaną genetycznie dysplazją szkieletu, do której objawów należy powstawanie zagęszczeń kostnych -szczególnie w okolicach przystawowych -widocznych na radiogramach w postaci cieni plamistych lub pasmowatych. Występowa-nie osteopoikilii wiąże się z mutacją genu LEMD3 odpowiedzialnego za gęstość mineralną kości. Gen ten wpływa także na ekspresję transformującego czynnika wzrostu β1 (transforming growth factor β1 -TGF-β1), który ma kluczowe znaczenie w procesie włóknienia oraz działanie immunosupresyjne i przeciwzapalne. Chorzy z osteopoikilią wykazują skłonność do nadmiernego bliznowacenia tkanek (bliznowce), obserwuje się u nich również występowanie chorób towarzyszących o podłożu autoimmunologicznym. W opisanym przypadku osteopoikilii towarzyszyło powstawanie bliznowców oraz objawy czterech chorób autoimmunologicznych: reumatoidalnego zapalenia stawów, miastenii, choroby Gravesa i Basedowa oraz niedokrwistości megaloblastycznej.

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Osteopoikilosis: report of a clinical case and review of the literature

Cited by 43 publications

References 18 publications

Benign spotted bones: a diagnostic dilemma

Benign spotted bones: a diagnostic dilemma

Abnormal Bone Scan in an Adult With Osteopoikilosis

Osteopoikilosis associated with keloids formation, rheumatoid arthritis, myasthenia, Graves` disease and megaloblastic anaemia

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