Erdheim-Chester Disease: A Rare Disease Causing Diagnostic Challenge in A 35 Year Old Male From Ethiopia

Authors

  • Abate Bane Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia. Author
  • Sebrina Ahmed Adera medical center, Addis Ababa, Ethiopia,Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia. Author
  • Fozia Abdela Adera medical center, Addis Ababa, Ethiopia,Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia. Author
  • Dawit Mamo Adera medical center, Addis Ababa, Ethiopia. Author
  • Tesfaye Kebede Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences,Department of Radiology, Addis Ababa, Ethiopia. Author
  • Yonas Girma Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences,Department of Pathology, Addis Ababa, Ethiopia. Author
  • Abdu Mohammed Adera medical center, Addis Ababa, Ethiopia. Author
  • Ahmed Adem Adera medical center, Addis Ababa, Ethiopia,Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia. Author
  • Tesfamariam Aklilu Adera medical center, Addis Ababa, Ethiopia,Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia. Author

DOI:

https://doi.org/10.47363/JCRR/2021(3)155

Keywords:

Erdheim-Chester Disease, Diagnostic Challenge

Abstract

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell tumor with histopathology features of foamy histocytes and fibrosis. The disease has varying manifestations from asymptomatic to life-threatening organ involvement including long bones, CNS, lung, heart, and kidneys. On histopathology staining, the cells exhibit CD68 positivity but CD1a and S100 negativity. The prognosis of the patients is poor. ECD poses a diagnostic and therapeutic challenge due to its resemblance to various disorders requiring a multidisciplinary approach. We report a case of a 35-year-old male patient from Ethiopia with literature review, who initially presented with massive chylous ascites and retroperitoneal manifestation but later developed pulmonary and cardiac manifestations. The diagnosis was suggested from an abdominal CT scan with subsequent image-guided biopsy which compartments a diagnosis. 

Author Biographies

  • Abate Bane, Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia.

    Abate Bane, Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia.

  • Sebrina Ahmed, Adera medical center, Addis Ababa, Ethiopia,Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia.

    Sebrina Ahmed, Adera medical center, Addis Ababa, Ethiopia,Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia.

  • Fozia Abdela, Adera medical center, Addis Ababa, Ethiopia,Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia.

    Fozia Abdela, Adera medical center, Addis Ababa, Ethiopia,Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia.

  • Dawit Mamo, Adera medical center, Addis Ababa, Ethiopia.

    Dawit Mamo, Adera medical center, Addis Ababa, Ethiopia.

  • Tesfaye Kebede, Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences,Department of Radiology, Addis Ababa, Ethiopia.

    Tesfaye Kebede, Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences,Department of Radiology, Addis Ababa, Ethiopia.

  • Yonas Girma, Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences,Department of Pathology, Addis Ababa, Ethiopia.

    Yonas Girma, Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences,Department of Pathology, Addis Ababa, Ethiopia.

  • Abdu Mohammed, Adera medical center, Addis Ababa, Ethiopia.

    Abdu Mohammed, Adera medical center, Addis Ababa, Ethiopia.

  • Ahmed Adem, Adera medical center, Addis Ababa, Ethiopia,Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia.

     Ahmed Adem, Adera medical center, Addis Ababa, Ethiopia,Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia.

  • Tesfamariam Aklilu, Adera medical center, Addis Ababa, Ethiopia,Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia.

    Tesfamariam Aklilu, Adera medical center, Addis Ababa, Ethiopia,Addis Ababa University, TikurAnbessa Specialized Hospital, Collage of Health Sciences, Department of Internal Medicine, Addis Ababa, Ethiopia.

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Published

2021-12-31

Issue

Vol. 3 No. 4 (2021): Volume 3 Issue 4

Section

Articles