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Melorheostosis
Leri’s Disease, Flowing Periosteal Hyperostosis
General Considerations
- One of a group of sclerosing bone disorders
- Rare
- Cause is unknown
- Produces thickening of the endosteum and periosteum
- Peak age of presentation is 5-20 years
- May be monostotic or polyostotic
- May involve one entire limb
- Usually does not involve multiple limbs
- Twice as common in lower extremities than elsewhere
Clinical Findings
- About 50% affected develop symptoms by age 20
- Adults present with
- Joint stiffness
- Deformity that may progress over time
- Children may present with
Imaging Findings
- Patterns described include:
- Candle-wax appearance (classic)
- Resembling myositis ossificans
- Resembling osteopathia striata
- Mixed
- Sclerotic lesions of cortical bones, usually in the diaphysis, that resemble “candle-wax-dripping”
- Cortical hyperostosis with an undulating appearance usually affecting one side of a bone
- Soft tissue lesions that may calcify
- Adjacent to involved bone
- May grow to compress nerves
- Usually low signal on MRI
- Bone scan is markedly positive
Differential Diagnosis
- Osteopathia striata
- Longitudinal dense striations
- Osteopoikilosis
- Punctate, rounded bone islands surrounding joints
- Osteosarcoma
Treatment
- Analgesia
- Nerve blocks
- Surgery directed at relieving contracture
Complications
- Associated soft tissue lesions and cutaneous lesions
- Vascular malformations
- Neurofibromatosis
- Tuberous sclerosis
- Hemangioma
- Muscle contractures
- Scoliosis
Prognosis
 
Melorheostosis. Frontal and oblique radiographs of the hand shown in close-up demonstrate the
undulating cortical hyperostosis representing the classical "candle-wax" dripping appearance
involving only the radial side of the 3rd metacarpal shaft.
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For this same photo without the annotations, click here and here
BoneTumor.org DeGroot, H.
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