Hand Cases to Consider.
Last updated Friday, February 11, 2005
Answer to case 1Diagnosis: Melorheostosis Melorheostosis was first described by Leri and Joanny in 1922. They
reported on a patient with "flowing hyperostosis" x-ray changes
resembling melting wax dripping down one side of a candle. Since then
many more cases have been reported, but the condition can still be
called uncommon.
The etiology of melorheostosis is unknown. It is not a hereditary
disease, but it appears to be congenital. The disease often manifests
itself in early childhood and is progressive. The changes are most
commonly seen in the lower limbs, but are also seen in the upper limbs.
Initial symptoms are pain, joint stiffness, fibrosis of skin and
deformities. Many of the children are first diagnosed as some form of
arthrogryposis because of the contractures and because the typical
skeletal changes often appear later. The disease is slowly progressive
with thickening of the cortex of the affected long bones, osteophyte
formation and secondary joint involvement. There does not appear to be
an increased risk of fracture and there are no reports of this
condition being premalignant.
The histological characteristics of melorheostosis is similar to
that of hyperostotic bone in other conditions such as osteopoikilosis.
There is a sclerotic, thickened and somewhat irregular laminae
surrounding the Haversian systems. The Haversian canals are of unequal
diameter. Inflammatory vascular changes have been reported.
Treatment of this rare disease is mostly conservative treatment of
symptoms. Surgical treatment is sometimes necessary for the joint
affection. Because the soft tissue changes frequently precede the
hyperostosis changes, specially in children, these patients are often
initially incorrectly diagnosed. References Campbell CJ, Papademetriou T, Bonfiglio M,: Melorheostosis: a case
report of the clinical, roentgenographic and pathological findings in
fourteen cases. J Bone Joint Surg 1968;50A:1281-304
Azuma H, Sakada T, Tanabe H, Handa M,: Melorheostosis of the hand: A report of two cases. J Hand Surg, 1992;17A:1076-8
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