Camurati-Engelmann disease. Effects of corticosteroids

Acta Clin Belg. 1998 Jun;53(3):189-92.

Abstract

Camurati-Engelmann disease is an uncommon condition, radiologically characterized by symmetric diaphyseal sclerosis involving the tubular bones. Clinical features include limb pain, muscle weakness, waddling gait and sometimes deafness. The evaluation is made by conjunction of radiographic and scintigraphic data. Corticosteroids and analgesics improve the quality of life, decrease the pain but do not alter the course of the disease. The evolution is unpredictable. The history of a 23 year old male with such a disorder is presented hereafter. His clinical course shows a very good response to the administration of corticosteroids, whereas no improvement is observed as far as the radiographic and isotopic features are concerned.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anti-Inflammatory Agents / therapeutic use*
  • Camurati-Engelmann Syndrome / complications
  • Camurati-Engelmann Syndrome / diagnostic imaging
  • Camurati-Engelmann Syndrome / drug therapy*
  • Diagnosis, Differential
  • Genetic Diseases, Inborn / diagnostic imaging
  • Genetic Diseases, Inborn / drug therapy*
  • Humans
  • Male
  • Pain / etiology
  • Pain Measurement
  • Quality of Life
  • Radiography
  • Steroids

Substances

  • Anti-Inflammatory Agents
  • Steroids