Introduction.

Erdheim Chester disease is a rare disorder of unclear etiology and variable age of presentation, characterized by abnormal proliferation of non-Langerhans histiocytes. It can present asymptomatic, with local manifestations or as systemic complications. The diagnosis is based on the clinical conditions of the patient, imaging studies, and the pathological study, through which the disease is confirmed.

Clinical case.

A male patient with a lytic lesion in the seventh costal arch, initially asymptomatic and later with persistent chest pain, underwent resection and reconstruction of the chest wall, with resolution of the symptoms and adequate postoperative evolution. The pathology report confirmed the diagnosis of Erdheim Chester’s disease.

Discussion.

The case of a patient with rib involvement and pathological fracture is presented as an unusual location and manifestation of Erdheim Chester’s disease, treated surgically appropriately, and without recurrence. Due to the diversity of symptoms that they can present and the involvement of multiple organs, in addition to imaging studies, the clinic should direct complementary tests such as electrocardiogram or echocardiogram, magnetic resonance imaging, or CT angiography. Expectant management has been recommended in asymptomatic patients, and medical treatment with glucocorticoids, BRAF inhibitors, and interferon alfa in symptomatic patients. The role of surgery has not been studied in depth.

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