Hairy kidney sign (Erdheim-Chester disease)

Last revised by Michael P. Hartung ◉ on 17 May 2022

Citation, DOI, disclosures and article data

Citation:

Sarangi P, Hartung M, Rasuli B, et al. Hairy kidney sign (Erdheim-Chester disease). Reference article, Radiopaedia.org (Accessed on 28 Mar 2025) https://doi.org/10.53347/rID-65541

DOI:

https://doi.org/10.53347/rID-65541

Permalink:

https://radiopaedia.org/articles/65541?embed_domain=fgbtvat.ebecbve.dbzabivdba.vdbabivdba.vdb

rID:

65541

Article created:

13 Jan 2019, Pradosh Kumar Sarangi

Disclosures:

At the time the article was created Pradosh Kumar Sarangi had no recorded disclosures.

View Pradosh Kumar Sarangi's current disclosures

Last revised:

17 May 2022, Michael P. Hartung ◉

Disclosures:

At the time the article was last revised Michael P. Hartung had the following disclosures:

Otsuka Pharmaceutical, Consultant (ongoing)
Innovenn, Inc, Consultant (ongoing)

These were assessed during peer review and were determined to not be relevant to the changes that were made.

View Michael P. Hartung's current disclosures

Revisions:

8 times, by 7 contributors - see full revision history and disclosures

Systems:

Urogenital

Sections:

Signs

The hairy kidney sign refers to the soft tissue ring of perirenal infiltration seen on cross-sectional imaging studies in Erdheim-Chester disease and is considered to be pathognomonic of this disease. The ‘‘hairy’’ description refers to the associated thickening of the bridging perirenal septa (Kunin septa) which are bands of fibrous tissue that extend between the renal capsule and the perirenal fascia ^1,2.