Erdheim-Chester Disease: The Effect of Bisphosphonate Treatment—A Case Report

• Published in: Archives of physical medicine and rehabilitation Vol. 86; no. 5; pp. 1053 - 1057
• Main Authors: Eyigör, Sibel, Kirazlı, Yeşim, Memis, Ayşenur, Başdemir, Gülçin
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.05.2005; Elsevier
• Subjects: Alendronate - therapeutic use; Biological and medical sciences; Bisphosphonates; Bones, joints and connective tissue. Antiinflammatory agents; Calcium Carbonate - therapeutic use; Case report; Densitometry; Diagnosis, Differential; Diphosphonates - therapeutic use; Diseases of the osteoarticular system; Drug Therapy, Combination; Erdheim-Chester Disease - diagnosis; Erdheim-Chester Disease - drug therapy; Female; Hematologic and hematopoietic diseases; Histiocytosis, non-Langerhans-cell; Humans; Medical sciences; Middle Aged; Osteoarthritis; Other diseases. Hematologic involvement in other diseases; Pharmacology. Drug treatments; Rehabilitation; Vitamin D - therapeutic use; Case report; Densitometry; Rehabilitation; Histiocytosis, non-Langerhans-cell; Bisphosphonates; Knee; Skin disease; Diseases of the osteoarticular system; Biological marker; Diphosphonic acid derivatives; Lipids; non-Langerhans-cell; Rehabilitation; Hemopathy; Lower extremity; Bisphosphonates; Case report; Densitometry; Histiocytosis; Sclerosis; Alendronic acid; Etiology; Degenerative disease; Diagnosis; Long bone; Chester-Erdheim disease; Human; Radiodiagnosis; Treatment efficiency; Exploration; Leg; Case study; Radiography; Chemotherapy; Treatment; Biopsy; Arthropathy; Osteoarthritis
• ISSN: 0003-9993; 1532-821X
• DOI: 10.1016/j.apmr.2004.05.005

Eyigör S, Kirazlı Y, Memis A, Başdemir G. Erdheim-Chester disease: the effect of bisphosphonate treatment—a case report. Erdheim-Chester disease is a distinctive pathologic and radiographic entity characterized by bilateral symmetric sclerosis of the diametaphyseal regions of long bones and infiltration of foamy lipid-laden histiocytes. It is a rare histiocytic disease of unknown etiology that is characterized pathologically by xanthogranulomatous infiltrates of multiple organs. We present a patient in her early sixties with bilateral mild knee and leg pain. The patient showed a typical bilateral symmetric medullary sclerosis at the diametaphyseal portions of long bones of the lower extremity. The diagnosis was confirmed by a bone biopsy, and bisphosphonate (alendronate, 70mg/wk) was given to the patient. After 9 months of treatment, biochemical markers of bone turnover, which were high at baseline, decreased to normal ranges. However, the radiographs showed that bone lesions had changed to lytic lesions. We propose use of bisphosphonates, such as alendronate, to decrease the biochemical markers of bone turnover. But we suggest that it is premature to conclude that bisphosphonates have any effect on lytic lesions and the progression of the disease as shown by changes in radiographs. Further studies with long-term follow-up and ultrastructural evaluation are needed.