U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Neutrophilic urticarial dermatosis

MedGen UID:
1773749
•Concept ID:
C5421655
•
Disease or Syndrome
HPO: HP:0033167

Definition

A neutrophilic interstitial, perivascular and/or perieccrine infiltrate with leukocytoclasia. Notably, leukocytoclastic vasculitis is not present. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNeutrophilic urticarial dermatosis

Professional guidelines

PubMed

Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients.
Gusdorf L, Asli B, Barbarot S, Néel A, Masseau A, Puéchal X, Gottenberg JE, Grateau G, Blanchard-Delaunay C, Rizzi R, Lifermann F, Kyndt X, Aubin F, Bessis D, Boye T, Gayet S, Rongioletti F, Sauleau E, Fermand JP, Lipsker D
Allergy 2017 Feb;72(2):177-182. Epub 2016 Sep 12 doi: 10.1111/all.13035. PMID: 27564982

Recent clinical studies

Etiology

Neutrophilic dermatoses.
Delaleu J, Lepelletier C, Calugareanu A, De Masson A, Charvet E, Petit A, Giurgea I, Amselem S, Karabina S, Jachiet M, Mahevas T, Ram-Wolff C, Vignon-Pennamen MD, Bagot M, Battistella M, Bouaziz JD
Rev Med Interne 2022 Dec;43(12):727-738. Epub 2022 Jul 20 doi: 10.1016/j.revmed.2022.06.007. PMID: 35870984
Lupus erythematosus: Significance of dermatologic findings.
Lenormand C, Lipsker D
Ann Dermatol Venereol 2021 Mar;148(1):6-15. Epub 2021 Jan 19 doi: 10.1016/j.annder.2020.08.052. PMID: 33483145
Schnitzler Syndrome: a Review.
Gusdorf L, Lipsker D
Curr Rheumatol Rep 2017 Aug;19(8):46. doi: 10.1007/s11926-017-0673-5. PMID: 28718061
Lupus erythematosus and neutrophilic urticarial dermatosis: a retrospective study of 7 patients.
Gusdorf L, Bessis D, Lipsker D
Medicine (Baltimore) 2014 Dec;93(29):e351. doi: 10.1097/MD.0000000000000351. PMID: 25546692Free PMC Article
Neutrophilic urticarial dermatosis: a variant of neutrophilic urticaria strongly associated with systemic disease. Report of 9 new cases and review of the literature.
Kieffer C, Cribier B, Lipsker D
Medicine (Baltimore) 2009 Jan;88(1):23-31. doi: 10.1097/MD.0b013e3181943f5e. PMID: 19352297

Diagnosis

Neutrophilic dermatoses.
Delaleu J, Lepelletier C, Calugareanu A, De Masson A, Charvet E, Petit A, Giurgea I, Amselem S, Karabina S, Jachiet M, Mahevas T, Ram-Wolff C, Vignon-Pennamen MD, Bagot M, Battistella M, Bouaziz JD
Rev Med Interne 2022 Dec;43(12):727-738. Epub 2022 Jul 20 doi: 10.1016/j.revmed.2022.06.007. PMID: 35870984
Lupus erythematosus: Significance of dermatologic findings.
Lenormand C, Lipsker D
Ann Dermatol Venereol 2021 Mar;148(1):6-15. Epub 2021 Jan 19 doi: 10.1016/j.annder.2020.08.052. PMID: 33483145
Neutrophilic urticarial dermatosis: an entity bridging monogenic and polygenic autoinflammatory disorders, and beyond.
Gusdorf L, Lipsker D
J Eur Acad Dermatol Venereol 2020 Apr;34(4):685-690. Epub 2019 Oct 28 doi: 10.1111/jdv.15984. PMID: 31562783
Neutrophilic urticarial dermatosis: A review.
Gusdorf L, Lipsker D
Ann Dermatol Venereol 2018 Dec;145(12):735-740. Epub 2018 Sep 14 doi: 10.1016/j.annder.2018.06.010. PMID: 30224079
Schnitzler Syndrome: a Review.
Gusdorf L, Lipsker D
Curr Rheumatol Rep 2017 Aug;19(8):46. doi: 10.1007/s11926-017-0673-5. PMID: 28718061

Therapy

Neutrophilic dermatoses with unusual and atypical presentations.
Feldmeyer L, Ribero S, Gloor AD, Borradori L
Clin Dermatol 2021 Mar-Apr;39(2):261-270. Epub 2020 Oct 16 doi: 10.1016/j.clindermatol.2020.10.012. PMID: 34272020
Neutrophilic urticarial dermatosis: A review.
Gusdorf L, Lipsker D
Ann Dermatol Venereol 2018 Dec;145(12):735-740. Epub 2018 Sep 14 doi: 10.1016/j.annder.2018.06.010. PMID: 30224079
Neutrophilic urticarial dermatosis and Sweet-like neutrophilic dermatosis: under-recognized neutrophilic dermatoses in lupus erythematosus.
Lee WJ, Kang HJ, Shin HJ, Won CH, Chang SE, Choi JH, Lee MW
Lupus 2018 Apr;27(4):628-636. Epub 2017 Oct 23 doi: 10.1177/0961203317736145. PMID: 29058993
Neutrophilic Epitheliotropism is a Histopathological Clue to Neutrophilic Urticarial Dermatosis.
Broekaert SM, Böer-Auer A, Kerl K, Herrgott I, Schulz X, Bonsmann G, Brehler R, Metze D
Am J Dermatopathol 2016 Jan;38(1):39-49. doi: 10.1097/DAD.0000000000000390. PMID: 26317390
The Schnitzler syndrome.
Lipsker D
Orphanet J Rare Dis 2010 Dec 8;5:38. doi: 10.1186/1750-1172-5-38. PMID: 21143856Free PMC Article

Prognosis

Lupus erythematosus: Significance of dermatologic findings.
Lenormand C, Lipsker D
Ann Dermatol Venereol 2021 Mar;148(1):6-15. Epub 2021 Jan 19 doi: 10.1016/j.annder.2020.08.052. PMID: 33483145
Neutrophilic urticarial dermatosis: an entity bridging monogenic and polygenic autoinflammatory disorders, and beyond.
Gusdorf L, Lipsker D
J Eur Acad Dermatol Venereol 2020 Apr;34(4):685-690. Epub 2019 Oct 28 doi: 10.1111/jdv.15984. PMID: 31562783
Neutrophilic urticarial dermatosis and Sweet-like neutrophilic dermatosis: under-recognized neutrophilic dermatoses in lupus erythematosus.
Lee WJ, Kang HJ, Shin HJ, Won CH, Chang SE, Choi JH, Lee MW
Lupus 2018 Apr;27(4):628-636. Epub 2017 Oct 23 doi: 10.1177/0961203317736145. PMID: 29058993
Schnitzler Syndrome: a Review.
Gusdorf L, Lipsker D
Curr Rheumatol Rep 2017 Aug;19(8):46. doi: 10.1007/s11926-017-0673-5. PMID: 28718061
Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients.
Gusdorf L, Asli B, Barbarot S, Néel A, Masseau A, Puéchal X, Gottenberg JE, Grateau G, Blanchard-Delaunay C, Rizzi R, Lifermann F, Kyndt X, Aubin F, Bessis D, Boye T, Gayet S, Rongioletti F, Sauleau E, Fermand JP, Lipsker D
Allergy 2017 Feb;72(2):177-182. Epub 2016 Sep 12 doi: 10.1111/all.13035. PMID: 27564982

Clinical prediction guides

Crohn disease-associated neutrophilic urticarial dermatosis: report and literature review of neutrophilic urticarial dermatosis.
Hou Z, Hinds BR, Cohen PR
Dermatol Online J 2017 Nov 15;23(11) PMID: 29447637
The Histopathology of Urticaria Revisited-Clinical Pathological Study.
Barzilai A, Sagi L, Baum S, Trau H, Schvimer M, Barshack I, Solomon M
Am J Dermatopathol 2017 Oct;39(10):753-759. doi: 10.1097/DAD.0000000000000786. PMID: 28858880
Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients.
Gusdorf L, Asli B, Barbarot S, Néel A, Masseau A, Puéchal X, Gottenberg JE, Grateau G, Blanchard-Delaunay C, Rizzi R, Lifermann F, Kyndt X, Aubin F, Bessis D, Boye T, Gayet S, Rongioletti F, Sauleau E, Fermand JP, Lipsker D
Allergy 2017 Feb;72(2):177-182. Epub 2016 Sep 12 doi: 10.1111/all.13035. PMID: 27564982
Mast-cell interleukin-1β, neutrophil interleukin-17 and epidermal antimicrobial proteins in the neutrophilic urticarial dermatosis in Schnitzler's syndrome.
de Koning HD, van Vlijmen-Willems IM, Rodijk-Olthuis D, van der Meer JW, Zeeuwen PL, Simon A, Schalkwijk J
Br J Dermatol 2015 Aug;173(2):448-56. Epub 2015 Jul 20 doi: 10.1111/bjd.13857. PMID: 25904179
Cryopyrin-associated periodic syndrome: an autoinflammatory disease manifested as neutrophilic urticarial dermatosis with additional perieccrine involvement.
Kolivras A, Theunis A, Ferster A, Lipsker D, Sass U, Dussart A, André J
J Cutan Pathol 2011 Feb;38(2):202-8. Epub 2010 Nov 10 doi: 10.1111/j.1600-0560.2010.01638.x. PMID: 21062341

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...