Resumo

CISNEROS BERNAL, Ernesto; GUTIERREZ RODRIGUEZ, Eulalio Alberto  e  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 16-Out-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.

Palavras-chave : Histiocytosis; histiocytosis of non-Langerhans cells; Erdheim-Chester disease.