Case Of the Week (COW) 10 March 2013
Answer:
Osteopoikilosis.
Discussion:
An Imaging auntminnie itself, its an uncommon osteosclerosing dysplasia with autosomal dominant penetrance and usually asymptomatic in nature. Associated cutaneous abnormalities are present, including dermatofibrosis lenticularis disseminata, keloid formation, and scleroderma-like lesions in 25% of the subjects.
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. The long axis of most of these lesions parallels that of the longitudinal axis of bones.
Differential diagnosis include blastic metastasis, tuberous sclerosis, and mastocytosis. However, to aid in the definitive diagnosis, a radiographic skeletal survey will demonstrate the symmetric juxtaarticular distribution and uniform size so characteristic of osteopoikilosis.
Reference:
Essentials of skeletal Radiology- Yochum & Rowe.
Contributed By:
Dr. Karunakaran M. Kalathi MD, Dr. Arun Chelladurai MD
Consultant Radiologists, Aarthi Scans, Thirunelveli, Tamilnadu, India
Answer:
Osteopoikilosis.
Discussion:
An Imaging auntminnie itself, its an uncommon osteosclerosing dysplasia with autosomal dominant penetrance and usually asymptomatic in nature. Associated cutaneous abnormalities are present, including dermatofibrosis lenticularis disseminata, keloid formation, and scleroderma-like lesions in 25% of the subjects.
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. The long axis of most of these lesions parallels that of the longitudinal axis of bones.
Differential diagnosis include blastic metastasis, tuberous sclerosis, and mastocytosis. However, to aid in the definitive diagnosis, a radiographic skeletal survey will demonstrate the symmetric juxtaarticular distribution and uniform size so characteristic of osteopoikilosis.
Reference:
Essentials of skeletal Radiology- Yochum & Rowe.
Contributed By:
Dr. Karunakaran M. Kalathi MD, Dr. Arun Chelladurai MD
Consultant Radiologists, Aarthi Scans, Thirunelveli, Tamilnadu, India