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melorheostosis(synonyms : candle bone disease , melting wax syndrome , leri disease ) is a rare chronic bone disorder , first described in 1922 by leri and joanny . men and women are equally affected , and no hereditary features have been discovered .
most common part of bone is diaphysis of the long bone of lower limb rarely the axial skeleton .
classical radiological appearance of flowing hyperosteosis resembling hardened wax that has dripped down the side of a candle .
a 35 years old woman presented with left leg pain with mild swelling and limitation of knee movement . on examination
plain radiographs showed extensive , dense , undulating or irregular cortical hyperostosis , resembling candle wax , extending along the length of bone .
only symptomatic treatment improve the condition of the patients , more fruitful result obtain with pamidronate and physiotherapy .
melorheostosis ( synonyms : candle bone disease , melting wax syndrome , leri disease ) , first described by leri and joanny in 1922 , is a rare benign sclerosing bone dysplasia .
melorheostosis is derives from the greek word melos = limb and rheos = flow due to the classic radiological appearance of  flowing hyperosteosis resembling hardened wax that has dripped down the side of a candle .
most common part of bone is diaphysis of the long bone of lower limb rarely the axial skeleton .
it may also present with limb - length discrepancy , joint stiffness , progressive deformity , ossification in adjacent soft tissues .
a 35-year - old woman presented with a history of left leg dull and aching pain with mild swelling and limitation of left knee movement .
plain radiographs [ fig 1 and 2 ] of left leg showed extensive , dense , undulating or irregular cortical hyperostosis , resembling candle wax , extending along the length of bone , resulting in deformity of the bone and narrowing of the medullary cavities .
laboratory findings for cbc , serum calcium , phosphorus , alkaline phosphatase , erythrocyte sedimentation rate , c - reactive protein , -fetoprotein , carcinoembryonic antigen were within normal limits .
treatment pamidronat 30 mg daily for 6 days as well as analgesic were given to the patient .
melorheostosis is a rare chronic bone disorder which was first described in 1922 by leri and joanny .
the onset of this rare diseases is insidious , and the first symptom is usually pain because of the sub periosteal bone formation .
skin changes may be seen in about 17% of cases and that may include hyperpigmentation of overlying skin and scleroderma .
melorheostosis mainly affects , the long bones of the upper and lower limbs , and also the short bones of the hand and foot , but rarely the axial skeleton .
melorheostosis may be associated with the tumours , including osteogenic sarcoma , malignant fibrous histiocytoma and dermoid tumours .
the monomelic variant is the most common presentation of this rare disease . in this patient ,
murray and mccredie1979 , hypothesis was that , embryonic infection of nerve root causes neural scarring and segmental bone sclerosis responsible for melorheostosis , because they detected a good correlation between hyperostotic lesions and the sclerotomes .
however , according to the hypothesis of murray and mccredie , when infection encroaches on nerve roots , the involved nerve roots should adjoin each other .
kim j - e demonstrated down regulation of adhesion proteins involved in osteoblastic regulation , specifically transforming growth factor  induced gene product , which may contribute to the development of hyperostosis and associated soft tissue abnormalities .
one possible etiology of melorheostosis is a loss of function mutation in the lemd3 gene ( 12q12 - 12q14.3 ) , a protein involved in bone morphogenic protein and tumor growth factor- signaling .
these two hypothesis support the genetic involment of this rare disease so further work have to be done , to find out exact cause and role of gene therapy for this disease .
it is associated with vascular malformations , soft tissue masses adjacent to the affected bone and scleroderma of the overlying skin .
histologic findings are usually nonspecific and often show dense bone formation , a mixture of mature and immature bone elements .
osteoclastic activity is not a prominent feature ; however , osteoblastic activity along the margins of osteons is common .
operative treatment consists of tendon lengthening , excision of hyperostotic bone , osteotomies , sympathectom y and amputation .
potential causes of the bone pain in melorheostosis include increased osteoclastic bone resorption and activation of pain receptors , raised intraosseous pressure and increased vascularity secondary to hyperosteosis and soft tissue involvement around joints .
thus , bisphosphonate treatment via a number of mechanisms would be expected to reduce inflammatory bone pain and symptoms in melorheostosis bisphosphonates inhibit osteoclast - mediated bone resportion by direct and indirect actions on osteoblasts and macrophages and bone vascularity . they have been shown to decrease bone pain , slow progression of bone lesion . the prognosis of a patient with melorheostosis is variable and depends on the anatomical location , extension into the soft tissues , and soft tissue changes .
the disease exhibits a slow , chronic course , with periods of exacerbation and arrest .
it is only diagnosed by characteristic candle wax type appearance in x - ray film .
only symptomatic treatment improve the condition of the patients , more fruitful result obtain with pamidronat and physiotherapy .
introduction : melorheostosis(synonyms : candle bone disease , melting wax syndrome , leri disease ) is a rare chronic bone disorder , first described in 1922 by leri and joanny . men and women are equally affected , and no hereditary features have been discovered . 
 onset is insidious , and most common symptom is pain . 
 most common part of bone is diaphysis of the long bone of lower limb rarely the axial skeleton . 
 classical radiological appearance of flowing hyperosteosis resembling hardened wax that has dripped down the side of a candle.case report : a 35 years old woman presented with left leg pain with mild swelling and limitation of knee movement . on examination 
 non tender bony heard swelling , hyperpigmented and restriction of knee movement present . 
 plain radiographs showed extensive , dense , undulating or irregular cortical hyperostosis , resembling candle wax , extending along the length of bone . 
 pamidronate as well asanalgesic were given to the patient . 
 physiotherepy started for the deformity.conclusion:routine laboratory findings usually are normal . 
 the exact cause remain unclear . 
 there is no definite treatment available for this disease . 
 only symptomatic treatment improve the condition of the patients , more fruitful result obtain with pamidronate and physiotherapy .