A 34-year-old woman presented with fever, central diabetes insipidus, xanthelasma palpebrarum, subcutaneous masses in the trunk, and long bone involvement. Based on skin and bone biopsies, she was diagnosed as Erdheim-Chester disease (ECD), one of the non-Langerhans cell histiocytoses. Despite administration of oral steroids and immunosuppressants as well as other treatments, little improvement was observed, and the subcutaneous masses gradually increased in size and number. Immunohistochemistry revealed that the infiltrating foamy histiocytes were diffusely positive for TNF-α. Therefore, we treated her with infliximab, an anti-TNF-α monoclonal antibody. After two administrations of infliximab, the subcutaneous masses decreased in size. Although the treatment strategy for ECD is still controversial, anti-TNF-α monoclonal antibody could be one of the therapeutic options.
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