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Title

MELORHEOSTOSIS, A RARE DISEASE - CASE REPORT

Background

The melorreostose is a rare sclerosing bone dysplasia, characterized by fluid hiperostosis the cortex of the tubular bones with radiology appearance of wax in dripping of unknown etiology. Only around 300 cases have been reported worldwide reaching equally men and women. It may affect a single bone (monostotic) or multiple bones (Polyostotic) and typically has a unilateral distribution. The most common sites are the long bones of the limbs and, rarely, the axial skeleton. The majority of the cases are asymptomatic. However, it may present insidious pain, muscular contracture, calcification of soft tissue, limitation of movement and discrepancies in the length of the members. The conservative treatment (bisphosphonates, NSAIDS, physical therapy) can improve the condition of patients.

Case report

A 68-year-old man, who hasn’t comorbidities or previous trauma, presented on his 30 years old, diffuse pain in the right lower limb, which worsened the ambulation. In 2004, sought to Rheumatology Service with radiograph showing areas of multiple sclerosis, lytic and blastic lesions associated in the right lower limb. There was suspicion of Paget's disease.
He presented painful sensitivity and increased heat in the previous surface of the right tibia, increase of soft parts in the right knee, discrepancy of MID < MIE: 4,3cm. The Laboratory exams were normal. The Radiographs of the right lower limb showed the changes described previously in two-thirds of the distal femur, patella, tibia and foot ipsilateral, with periosteal and endosteal cortical thickening, compatible with characteristic aspect of “dripping” in candle (Fig .1). Bone scintigraphy with hypercaptation diffuse, heterogeneous, involving the same sites .
The patient received symptomatic treatment with non-steroidal anti-inflammatory drugs and started bisphosphonates. Despite the persistence of radiological findings, It remains with good control of painful symptoms.

Conclusion

The melorheostosis is a rare disease, often asymptomatic. The possible causes of pain stem from an increased bone resorption, with stimulation of pain receptors and increased local bone vascularization, leading to an increase of the intramedullary pressure. The radiography method is sufficient for the diagnosis. There is no definitive treatment available for this disease, but the bisphosphonates have been used as an alternative for the control of bone metabolism and reduction of pain. In our patient, the therapy instituted proved efficient in the management of subjective symptoms.

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