THORACIC SPINE MELORHEOSTOSIS

THORACIC SPINE MELORHEOSTOSIS

OGAWA, GABRIELA MIYUKI TEODORO; BELLINI, PAMELLA PAULA; ANDRADE, SAMUEL OLIVEIRA; BONFIM, LARA RIBEIRO TEIXEIRA; PUGLIESI, ALISSON ALIEL VIGANO; GAINO, JULIANA ZONZINI; COIMBRA, IBSEN BELLINI

Pôster:

Originally described by Leri and Joanny in 1922, melorheostosis is a rare, congenital, non-hereditary disease of unknown etiology. It is a mixed mesodermal sclerosing bone dysplasia that predominantly affects intramembranous ossification. Both cortical bone (hyperostosis) and adjacent soft tissue structures (sclerosis) are affected, with long bones being the most affected. The main clinical manifestation is localized chronic pain, although the disease may be asymptomatic. The diagnosis can be made, in most cases, by simple radiography with the characteristic image similar to the melted wax flowing on the margin of a candle. The purpose of the report is to present the case of a patient with polyostotic involvement of melorheostosis, including involvement of the vertebral column, a rare manifestation of the disease.

Case report

MCR, female, 55 years old with diagnosis of melorheostosis after radiographs requested for investigation of arthralgia of the upper and lower limbs, accompanied by pain and prolonged morning stiffness of the thoracic spine (e.g. figure1). In addition to involvement of the skull, knees (e.g. figure 2), feet (e.g. figure 3), femur, hand (e.g.figure 4) and elbows, thoracic spine involvement was also observed. Initially treated with alendronate 140mg / week, without response, it was replaced by zolendronic acid, with the exchange resulting in improvement of the algic symptoms.

Conclusion

The case aims to illustrate the possibility of multiple sites involvement by melorheostosis, and it is important to investigate less commonly affected sites.

Originally described by Leri and Joanny in 1922, melorheostosis is a rare, congenital, non-hereditary disease of unknown etiology. It is a mixed mesodermal sclerosing bone dysplasia that predominantly affects intramembranous ossification. Both cortical bone (hyperostosis) and adjacent soft tissue structures (sclerosis) are affected, with long bones being the most affected. The main clinical manifestation is localized chronic pain, although the disease may be asymptomatic. The diagnosis can be made, in most cases, by simple radiography with the characteristic image similar to the melted wax flowing on the margin of a candle. The purpose of the report is to present the case of a patient with polyostotic involvement of melorheostosis, including involvement of the vertebral column, a rare manifestation of the disease.

Case report

MCR, female, 55 years old with diagnosis of melorheostosis after radiographs requested for investigation of arthralgia of the upper and lower limbs, accompanied by pain and prolonged morning stiffness of the thoracic spine (e.g. figure1). In addition to involvement of the skull, knees (e.g. figure 2), feet (e.g. figure 3), femur, hand (e.g.figure 4) and elbows, thoracic spine involvement was also observed. Initially treated with alendronate 140mg / week, without response, it was replaced by zolendronic acid, with the exchange resulting in improvement of the algic symptoms.

Conclusion

The case aims to illustrate the possibility of multiple sites involvement by melorheostosis, and it is important to investigate less commonly affected sites.

Palavras-chave:

DOI: 10.5151/sbr2019-290

Referências bibliográficas

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Como citar:

OGAWA, GABRIELA MIYUKI TEODORO; BELLINI, PAMELLA PAULA; ANDRADE, SAMUEL OLIVEIRA; BONFIM, LARA RIBEIRO TEIXEIRA; PUGLIESI, ALISSON ALIEL VIGANO; GAINO, JULIANA ZONZINI; COIMBRA, IBSEN BELLINI; "THORACIC SPINE MELORHEOSTOSIS", p-290-290. In: Anais do 36º Congresso Brasileiro de Reumatologia. [ISBN 978-85-212-1892-0]. São Paulo: Blucher, 2019.
ISSN 23577282, DOI 10.5151/sbr2019-290