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2019, Number 2

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Rev Fac Med UNAM 2019; 62 (2)

Incidental diagnosis of Erdheim-Chester disease in a patient with unusual presentation

Cisneros BE, Gutiérrez REA, Soto SLG

Language: Spanish
References: 23
Page: 22-30
PDF size: 323.35 Kb.


ABSTRACT

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.


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