DOI: 10.36347/sjmcr.2023.v11i01.005

Pages: 16-19

The sign of the scalp is the presence of perirenal fibrosis classically recognized as pathognomonic of Erdheim- Chester disease, a rare non-Langheransian histiocytosis. We report the case of a 31-year-old woman, wrongly followed and treated for bilateral polycystic kidney disease. She had no other medico-surgical history and had presented abdominal pain accentuated at the lumbar level, revealing on the abdomino-pelvic computed tomography performed at the ''Marie Curie'' Medical Clinic in Bamako, Mali, an aspect of hairy kidney. The diagnosis of non-Langheransian histiocytosis was retained in view of this specific and pathognomonic aspect. This was, to our knowledge, the first case described in Mali, the purpose of this work being to illustrate the role of CT in the diagnostic management of Erdheim- chester disease and to draw the attention of young radiologists and other specialists on the pathognomonic aspect of the scalp. The appearance of a scalp being considered semiologically as a very suggestive sign of Erdheim-Chester disease, the radiologist and his urologist or nephrologist counterparts must recognize these signs and finally not wrongly treat patients with this rare pathology.