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Erschienen in:
14.11.2016 | Classics in Abdominal Imaging
The “hairy kidney” sign
Erschienen in: Abdominal Radiology | Ausgabe 3/2017
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While some hair has historical precedent in Radiology (Fig. 1), the “hairy kidney” describes a soft tissue rind of perirenal infiltration seen on cross-sectional imaging studies. The appearance is considered nearly pathognomonic of Erdheim–Chester disease (ECD), a rare non-Langerhans histiocytosis [1]. The “hairy” description refers to the associated thickening of the perirenal septa—bands of fibrous tissue that extend between the renal capsule and the renal fascia (Fig. 2) [2]. Perirenal involvement has been reported in up to 68% of ECD cases [3]. Other pathologic processes that can produce similar findings include retroperitoneal fibrosis, perirenal lymphoma, and amyloidosis [4].
Fig. 1
While some things in radiology are expected to be hairy, the kidneys are not. a Wilhelm Conrad Röntgen, ca 1900, from Wikimedia Commons (commons.wikimedia.org). b 21st century doppelgänger Christopher McAdams, Radiology Resident at Wake Forest School of Medicine, Class of 2017 (with permission), at ACR 2016, photo and concept courtesy of Lauren Parks Golding
Fig. 2
a Axial and b coronally reconstructed CT images showing extensive perirenal infiltration and perirenal septal thickening in a case of Erdheim–Chester disease. Also note the “coated aorta,” with circumferential perivascular infiltration about the thoracoabdominal aorta
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