Services on Demand
Journal
Article
Indicators
Cited by SciELO
Access statistics
Related links
Similars in SciELO
Share
Revista de la Facultad de Medicina (México)
On-line version ISSN 2448-4865Print version ISSN 0026-1742
Abstract
CISNEROS BERNAL, Ernesto; GUTIERREZ RODRIGUEZ, Eulalio Alberto and SOTO SALAZAR, Laura Gabriela. Incidental diagnosis of Erdheim-Chester disease in a patient with unusual presentation. Rev. Fac. Med. (Méx.) [online]. 2019, vol.62, n.2, pp.22-30. Epub Oct 16, 2020. ISSN 2448-4865. https://doi.org/10.22201/fm.24484865e.2019.62.2.05.
Erdheim-Chester disease (ECD) is a rare presentation of non-Langerhans cell histiocytosis, which affects adults that are between 50 and 70 years old. The diagnosis is confirmed by clinical, radiological and histopathological manifestations and immunohistochemistry markers (CD68 (+), CD1a (-) and S100 with variable behavior). It is a rare disease of which only about 600 cases have been reported, whose main clinic is characterized by bone involvement and general symptoms. It has variable severity and prognosis depending on the organic commitment. We present a clinical case of a 45-year-old male patient who underwent histopathological and incidental immunohistochemical diagnosis of Erdheim-Chester disease after presenting spontaneous splenic rupture without any other documented condition, this is an unusual presentation of this rare disease. An updated review of the subject and the diagnostic criteria of the ECD is being performed.
Keywords : Histiocytosis; histiocytosis of non-Langerhans cells; Erdheim-Chester disease.