On Osteopoikilosis

1. OsteopoikilosisSynonyms include osteopathia
condensans disseminata and spotted bones.
DR MADAN's Slide
Dr MADAN MOHAN Consultant, Orthopaedics, KIMS, Trivandrum

2. •First described by Albers-Schönberg and by
Ledoux-Lebard and Chabaneux in the early
1900s
•Characterized by small round or ovoid
radiopacities appearing in the juxtaarticular
regions of bone.
•It has been described as autosomal
dominant in transmission
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3. Clinical Features
•Males are affected more frequently than
females.
•In approximately 25% of cases cutaneous
abnormalities are present, including
dermatofibrosis lenticularis disseminata, keloid
formation, and scleroderma-like lesions
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4. Pathology
• The lesions may represent foci of bone that failed to
become cancellous during the course of growth and
differentiation.
• It has been suggested that osteopoikilosis, because of
its diffuse nature, hereditary mode of transmission,
and association with cutaneous abnormalities, may be
a manifestation of a metabolic connective tissue
disorder
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5. Radiology
• Multiple small radiopacities found scattered in epiphyseal and
metaphyseal regions are generally pathognomonic.
• The lesions are symmetric, with a predilection for the long tubular
bones, carpals, and tarsals
• In the pelvis and scapula the densities are found adjacent to the
acetabulum and glenoid.
• Rarely, lesions are found in the skull, spine, ribs, and clavicles.
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11. •Osteopoikilosis has been found in association
with both osteopathia striata and
melorheostosis, and it has been suggested that
these are related conditions.
•Osteopoikilosis and melorheostosis may be the
result of mutations at the same genetic locus
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12. Differential Diagnosis
The considerations for
differential diagnosis include
blastic metastasis, tuberous
sclerosis, and mastocytosis
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