Neutrophilic urticarial dermatosis: A review

Ann Dermatol Venereol. 2018 Dec;145(12):735-740. doi: 10.1016/j.annder.2018.06.010. Epub 2018 Sep 14.

Abstract

Neutrophilic urticarial dermatosis (NUD) is a rare form of dermatosis. In clinical terms, it consists of a chronic or recurrent eruption comprising slightly elevated, pink to reddish plaques or macules. The elementary lesion lasts 24 to 48hours and resolves without leaving any residual pigmentation. Extra-cutaneous signs are common, particularly fever or arthralgia. At histopathology, the dermis contains dense neutrophilic interstitial infiltrate with leukocytoclasis, but without fibrinoid necrosis of vessel walls. NUD often occurs in a setting of underlying systemic disease. The most commonly associated diseases are adult-onset Still's disease, Schnitzler syndrome, lupus erythematosus and cryopyrin-associated periodic syndromes. Treatment of NUD depends on the clinical context. Dapsone and colchicine are often effective.

Keywords: Adult-onset Still's disease; Dermatose neutrophilique; Dermatose neutrophilique urticarienne; Lupus erythematosus; Lupus érythémateux; Maladie de Still de l’adulte; Neutrophilic dermatosis; Neutrophilic urticarial dermatosis; Schnitzler syndrome; Syndrome de Schnitzler; Urticaire; Urticaria.

Publication types

  • Review

MeSH terms

  • Arthralgia / etiology
  • Colchicine / therapeutic use
  • Cryopyrin-Associated Periodic Syndromes / complications
  • Dapsone / therapeutic use
  • Dermis / pathology
  • Diagnosis, Differential
  • Drug Eruptions / pathology
  • Drug Resistance
  • Fever / etiology
  • Histamine Antagonists / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lupus Erythematosus, Systemic / complications
  • Neutrophils / pathology*
  • Schnitzler Syndrome / complications
  • Still's Disease, Adult-Onset / complications
  • Sweet Syndrome / diagnosis
  • Urticaria / diagnosis
  • Urticaria / drug therapy
  • Urticaria / etiology
  • Urticaria / pathology*
  • Vasculitis / diagnosis

Substances

  • Histamine Antagonists
  • Immunosuppressive Agents
  • Dapsone
  • Colchicine