Open AccessErdheim-Chester disease. Series of nineteen cases and a relevant differential diagnosis
Author(s) -
A. Reibaldi,
Lorena Sager,
Romina Calvo,
Jésica Gallo,
Alberto Ortiz,
Susana Roverano,
Diego Baena,
Francisco Caeiro,
G. Robai,
Sergio Paira
Publication year - 2020
Publication title -
revista argentina de reumatología
Language(s) - English
Resource type - Journals
eISSN - 2362-3675
pISSN - 0327-4411
DOI - 10.47196/rar.v31i2.406
Subject(s) - erdheim–chester disease , medicine , histiocytosis , asymptomatic , differential diagnosis , histopathology , disease , nodule (geology) , radiology , pathology , dermatology , paleontology , biology
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.