It is rarely associated with other sclerosing bone dysplasias such as melorheostosis
Radiologically, it is characterized by the presence of hyperostosis foci at any point in the skeleton, and associated with numerous disorders including Buschke-Ollendorff syndrome, rheumatoid arthritis, scleroderma, ankylosing spondylitis, synovial osteochondromatosis, Klippel-Feil syndrome, cleft palate, Familial Mediterranean fever, endocrine diseases, syndactyly, and melorheostosis
. (4) The foci of hyperostosis are due to alteration in the maturation of the endochondral bone (impairment of secondary spongiosa resorption and remodeling), which is usually detected between 20-30 years of age.
The rare disorder, known as melorheostosis
, causes excess bone formation that resembles dripping candle wax on x-rays.
is a rare, nonhereditary, benign, mesenchymal condition of unknown aetiology affecting the bones and surrounding tissues .
99mTc-pyrophosphate bone imaging in osteopoikilosis, osteopathia striata, and melorheostosis
. Radiology 1978; 127:439-43.
Osteopoikilosis had also been associated with nanism, spinal stenosis, dystocia , organ anomanies such douplicated ureter, aorta coarctation, endocrine disorders, disc protrusions, melorheostosis
and tuberous sclerosis [22, 28].
Leri is associated with Melorheostosis
, "candle bone disease", a rare disorder of bone (Leri-Joanny syndrome), Leri- J-E Weill syndrome (a skeletal dysplasia combining dorsal subluxation of the distal end of the ulna (Madelung deformity) with mesomelic short stature), and Leri sign (a pyramidal sign, or joint reflex seen in spastic hemiplegia) and Leri's disease-paroxysmal pain precipitated by exposure to cold and high humidity.
INTRODUCTION: Two French neurologists, Leri and Joanny (1922) published the first case in which an arm was affected, and suggested the descriptive title melorheostosis
The other deferred microscope slide review required correlation with radiographic imaging to rule out melorheostosis
. Five of the digital image reviews were deferred.
Acquired macrodactyly includes dactylitis secondary to infection, infarction, and Still's disease; osteoid osteoma and melorheostosis
and can be distinguished from the congenital type by the appearance at birth.
Increased bone density without modifcation of bone shape Osteopetrosis precocious type Generalised AR delayed type Type 1 uniform AD intermediate type Type 2 endobones with renal tubular Generalised AR AR acidosis Similar other types Axial osteosclerosis osteomesopyknosis Focal sclerosis AD AR with bamboo hair in vertebrae/pelvis Pycnodysostosis Generalised AR Osteosclerosis Cortical AD Stanesu type thickening of long bones, defcient facial sinus development Osteopathia striata Radiodense SP XLD isolated with striations on all cranial sclerosis bones with cranial sclerosis Sponastrime Striated AR dysplasia metaphysis Melorheostosis
Flowing SP hyperostosis Osteopoikilosis Radiodense spots AD Mixed sclerosing Combined pattern SP bone dysplasia 2.
These features are pathognomonic of melorheostosis
, a rare hyperostotic condition that is distinguished by a linear pattern of distribution along the axes of long bones .