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Revista argentina de reumatología

Print version ISSN 0327-4411On-line version ISSN 2362-3675


REIBALDI, Alejandro et al. Erdheim-Chester Disease: Series of nineteen cases and a relevant differential diagnosis. Rev. argent. reumatolg. [online]. 2020, vol.31, n.2, pp.31-36. ISSN 0327-4411.

Erdheim-Chester disease (ECD) is a non-Langerhans histiocytosis, protein manifestations at start and little known. We included 19 patients from June 2012 to June of 2019. Inclusion criteria: clinical features, histopathology and immunostaining compatible with ECD. We excluded patients with undefined features. Results: Bones were the most frequent affected, half of them were asymptomatic. Seventy per cent of the patients were women, and 7 of them developed a nodule breast as first manifestation of ECD. The patients were treated with corticosteroids associated or not with immunosuppressants. The mortality rate was 16%. Conclusion: We reported a series of patients with ECD, enhancing the most frequent features. It is striking the number of patients with breast involvement; we propose to include the Erdheim-Chester disease in differential diagnosis of breast tumor.

Keywords : Erdheim-Chester disease; histiocytosis; foamy macrophages; giant cells; xanthomas; xanthogranulomas; osteoesclerosis.

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