Exophthalmos, low back pain and hypertension: a report of one case with literature review / 中华风湿病学杂志

ABSTRACT

Objective By analyzing the clinical manifestations and pathologic features of Erdheim-Chester's disease (ECD) to improve the recognition of the disease.Methods The clinical characteristics,diagnosis and treatment of a young male with ECD were reported and the related literature was reviewed.Results A previously healthy young male patient with bilateral exophthalmos,blurred vision of right eye,polyuria and hypertension without obvious causes for nine months were admitted into our hospital.He developed low back pain two months ago.Thoracic vertebra Magnetic resonance imaging (MRI) showed multiple nodules extending from the 2th-7th thoracic vertebrae intra-medullary.MRI of the brain showed multiple masseswith abnormal intensities within the retro-ocular intraconal muscle cone,sellar and cavernous sinus,maxillary sinus.Biopsy specimens from the right orbital lesion demonstrated proliferation of fibrous connective tissue and fat tissue infiltrating with lymphocytes,plasma cells,eosinophils,foam cells,spindle cells,and multinucleated giant cells accompanied by fat necrosis.Immunohistochemistry showed infiltrated lymphocytes stained positive for CD68,CD20,CD3,LCA and negative for CD1a,S-100 protein and langerin.The clinical symptoms of exophthalmos and low back pain relieved after treated with methylprednisolone and interferon-α.Conclusion Understanding the characteristics of ECD can help to make the correct diagnosis and treatment.