Ribbing disease (multiple diaphyseal sclerosis): imaging and differential diagnosis

AJR Am J Roentgenol. 1996 Sep;167(3):689-94. doi: 10.2214/ajr.167.3.8751682.

Abstract

Objective: This study describes the clinical presentation and the course of Ribbing disease in six patients and illustrates imaging features on plain radiography, conventional and computed tomography, and 99mTc-methylene diphosphonate bone scans.

Materials and methods: Between 1982 and 1990, six female patients presented with painful bony lesions that were believed to be Ribbing disease. Ten bones were affected: both tibiae in three patients, a unilateral tibia in one, both femora in one, and a unilateral femur in one. Plain radiographs and either conventional or computed tomography were available for all patients and 99mTc-methylene diphosphonate bone scans, for five patients. All patients underwent open biopsy and/or surgical decompression.

Results: The diagnosis was reached in all patients through a combination of clinical findings (lack of systemic signs of infection or laboratory values suggesting metabolic bone disease), imaging, histologic evaluation, and specimen cultures. Radiographs and tomographic studies showed benign-appearing endosteal and periosteal cortical thickening. Intense uptake of radionuclide tracer was confined to the shaft of all involved bones. All pathologic specimens revealed nonspecific changes that included a slow increase in the mass of cortical and endosteal bone. These specimens also assisted in excluding neoplastic or infectious causes for the new bone formation.

Conclusion: Ribbing disease is a rare disorder that, on imaging studies, may simulate stress fracture, chronic infection, bone-forming neoplasia, or a systemic metabolic or endocrine disorder. Clinical and imaging features may suggest the correct diagnosis.

MeSH terms

  • Adult
  • Camurati-Engelmann Syndrome / diagnosis
  • Diagnosis, Differential
  • Diaphyses / pathology
  • Female
  • Femur / diagnostic imaging
  • Femur / pathology
  • Humans
  • Osteosclerosis / diagnosis*
  • Radionuclide Imaging
  • Technetium Tc 99m Medronate
  • Tibia / diagnostic imaging
  • Tibia / pathology
  • Tomography, X-Ray Computed

Substances

  • Technetium Tc 99m Medronate