Neutrophilic Urticarial Dermatosis

  • Laurence Gusdorf
  • Dan LipskerEmail author


Neutrophilic urticarial dermatosis (NUD) was first described in 2009 by one of us [1] as an eruption consisting of rose or red macules or slightly elevated plaques vanishing within 24 h. The histopathologic findings are a dense perivascular and interstitial infiltrate of neutrophils with leucocytoclasia but without vasculitis.


  1. 1.
    Kieffer C, Cribier B, Lipsker D. Neutrophilic urticarial dermatosis: a variant of neutrophilic urticaria strongly associated with systemic disease. Report of 9 new cases and review of the literature. Medicine (Baltimore). 2009;88:23–31.CrossRefGoogle Scholar
  2. 2.
    Broekaert SMC, Boer-Auer A, Kerl K, Herrgott I, Schulz X, Bonsmann G, et al. Neutrophilic epitheliotropism is a histopathological clue to neutrophilic urticarial dermatosis. Am J Dermatopathol. 2016;38:39–49.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Gusdorf L, Bessis D, Lipsker D. Lupus erythematosus and neutrophilic urticarial dermatosis: a retrospective study of 7 patients. Medicine (Baltimore). 2014;93:e351.CrossRefGoogle Scholar
  4. 4.
    Kolivras A, Theunis A, Ferster A, Lipsker D, Sass U, Dussart A, et al. Cryopyrin-associated periodic syndrome: an autoinflammatory disease manifested as neutrophilic urticarial dermatosis with additional perieccrine involvement. J Cutan Pathol. 2011;38:202–8.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Simon A, Asli B, Braun-Falco M, De Koning H, Fermand J-P, Grattan C, et al. Schnitzler’s syndrome: diagnosis, treatment, and follow-up. Allergy. 2013;68:562–8.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Gusdorf L, Asli B, Barbarot S, Neel A, Masseau A, Puechal X, et al. Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients. Allergy. 2017;72:177–82.CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Lipsker D. The need to revisit the nosology of cutaneous lupus erythematosus: the current terminology and morphologic classification of cutaneous LE: difficult, incomplete and not always applicable. Lupus. 2010;19:1047–9.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Herbert VG, Ahmadi-Simab K, Reich K, Boer-Auer A. Neutrophilic urticarial dermatosis (NUD) indicating Cryopyrin-associated periodic syndrome associated with a novel mutation of the NLRP3 gene. J Eur Acad Dermatol Venereol. 2016;30:852–3.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992;19:424–30.PubMedPubMedCentralGoogle Scholar
  10. 10.
    Lee JY-Y, Yang C-C, Hsu MM-L. Histopathology of persistent papules and plaques in adult-onset Still’s disease. J Am Acad Dermatol. 2005;52:1003–8.CrossRefPubMedGoogle Scholar
  11. 11.
    Sun NZ, Brezinski EA, Berliner J, Haemel A, Connolly MK, Gensler L, et al. Updates in adult-onset Still disease: atypical cutaneous manifestations and associations with delayed malignancy. J Am Acad Dermatol. 2015;73:294–303.CrossRefPubMedGoogle Scholar
  12. 12.
    Nguyen CV, Miller DD. Serum sickness-like drug reaction: two cases with a neutrophilic urticarial pattern. J Cutan Pathol. 2017;44:177–82.CrossRefPubMedGoogle Scholar
  13. 13.
    De Koning HD, van Gijn ME, Stoffels M, Jongekrijg J, Zeeuwen PLJM, Elferink MG, et al. Myeloid lineage-restricted somatic mosaicism of NLRP3 mutations in patients with variant Schnitzler syndrome. J Allergy Clin Immunol. 2015;135(2):561–4.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Service de DermatologieHôpitaux Universitaires de StrasbourgStrasbourgFrance

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