What we see is hyperostosis of the cortical bone of the left humerus. It is benign in appearance. No soft tissue mass, no abnormal periosteal reaction or destructive changes. This is most consistent with melorheostosis.
- Classically described as flowing hyperostosis
- Dripping candle wax sign
- Monostotic (single bone), monomelic (single limb), or polyostotic (multiple bones) - but often on a single side of body even when polyostotic
- Often follows a sclerotome
- Irregular thickening of cortical bone (cortical hyperostosis)
- Rare sclerosing bone dysplasia
- Patient's often discovered incidentally when looking for something else
- When symptomatic - often doesn't present till late teens early adulthood - limb stiffness and pain
- Can progress to disability even amputation.
- Can cause muscle contractures, tendon and ligament shortening and soft tissue involvement; growth disturbances (increased width, angulation, unequal limb length)
- Soft tissue - tense, erythematous and shiny skin; anomalous pigmentation; induration and edema of subq tissues; fibrosis; weakness and atrophy of muscles; and linear scleroderma
- Predominately limbs with lower more common than upper extremity
- Also can be seen in skull, facial bones, ribs and vertebrae
- If clavicle, scapula, or pelvis - adjacent limb usually also involved
- Can have endosteal hyperostosis as well
- Can appear like osteopoikilosis - esp in tarsal and carpal areas
- Pelvis and scapula (flat bones) - may appear radiating or localized sclerotic patches
- 25% have soft tissue calc and/or ossification
- Can be associated with linear scleroderma, osteopoikilosis, osteopathia striata, Neurofibromatosis, tuberous sclerosis, hemangiomas
- Have also been associated with glomus tumors and AVMs
- 5 basic imaging patterns - classic, osteoma-like, myositis-ossificans like, osteopathia striata like, mixed
References - http://radiology.rsnajnls.org/cgi/content/full/246/2/638 ; Bone and Joint Imaging (Resnick - i.e. Baby Resnick)
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