Rare Case of Urethral Stricture Presentation in Erdheim-Chester Disease

الملخص

Erdheim-Chester disease is multisystemic involvement of almost all organs and tissues of the body by excessive production of white blood cells by histiocytes. This present case showed the involvement of the urological system in bilateral swollen kidneys, which was seen in both ultrasound as well as contrast computed tomography. Renal parameters and urine culture were normal. Uroflowmetry showed signs of stricture, which was confirmed through retrograde urethrography. There were no clinical signs of other systemic involvement. Radiological examinations were conducted, which showed asymptomatic involvement of sclerosis in bilateral knees. To confirm, further bone biopsy was performed, which had foamy histocyte infiltration along with Touton-like giant cells on immunohistochemistry. Though the prognosis of this disease is poor, early diagnosis and alleviating symptoms require accurate judgment by clinicians in multidisciplinary teamwork.