CASE REPORT


Solitary Radiolucent Erdheim-chester Disease: A Case Report and Literature Review



Aleksandar Popovic1, Christopher Curtiss2, Timothy A. Damron3, *
1 College of Medicine, SUNY Upstate Medical University, NY 13057, United States
2 Department of Pathology, SUNY Upstate Medical University, NY 13057, United States
3 Department of Orthopedic Surgery, SUNY Upstate Medical University, NY 13057, United States


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Creative Commons License
© 2021 Popovic et al.

open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: https://creativecommons.org/licenses/by/4.0/legalcode. This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

* Address correspondence to this author at the Department of Orthopedic Surgery,SUNY Upstate Medical University, 6620 Fly Road, Suite 200, East Syracuse, New York 13057, United States; Tel: 315-464-4472; E-mail: damront@upstate.edu


Abstract

Background:

Erdheim-chester disease (ECD) is a rare non-Langerhans histiocytosis of unknown etiology, which typically presents with bilateral symmetric osteosclerosis and multi-organ involvement. Lesions may be intraosseous or extraosseous and involve the heart, pulmonary system, CNS, and skin in order of decreasing likelihood.

Objective:

The objective of this study is to discuss a case of erdheim-chester disease and conduct a review of the literature.

Case:

We describe a rare case of erdheim-chester in an asymptomatic 37-year-old male who was diagnosed after suffering a right ulnar injury. Subsequent evaluation revealed a solitary radiolucent ulnar lesion without multi-system involvement.

Results & Conclusion:

The case is unique in its solitary distribution, lytic radiographic appearance, and asymptomatic presentation preceding pathologic fracture. This presentation may simulate multiple other bone lesions.

Keywords: MRI, erdheim-chester disease, Asymptomatic, Unilateral, Periaortic fibrosis, Lesion.