title: Neutrophilic urticaria with systemic inflammation: a case series.
abstract: OBJECTIVE
  Predominantly neutrophilic infiltrates are seen in a subset of patients with urticaria. The lesions tend to be less itchy and poorly responsive to standard therapy, including antihistamines. We describe 2 patients having neutrophilic urticaria with systemic inflammation (NUSI) without known connective tissue disorder or malignancy. We propose the term NUSI to help classify a previously undefined multisystemic inflammatory entity likely mediated by interleukin 1 (IL-1).
  
  
  METHODS
  Patient 1, a 47-year-old woman, was seen with urticaria and associated night sweats, fevers, and polyarticular arthritis. Acute-phase reactants were elevated with worsening of symptoms. Initial treatment with a combination of topical and systemic corticosteroids, antihistamines, and immunosuppressants was unsuccessful. A 100% clinical resolution was achieved with anakinra, an IL-1 receptor antagonist. Patient 2, a 24-year-old woman, was seen with urticaria and associated joint pain and swelling. Initial treatment included a combination of antihistamines, colchicine, and dapsone. Only colchicine provided moderate benefit but was stopped because of significant gastrointestinal tract discomfort. Anakinra was initiated; the patient achieved 100% control while receiving daily therapy.
  
  
  CONCLUSIONS
  The diagnosis of NUSI is important to consider in patients who are seen with antihistamine-resistant urticaria in combination with systemic inflammatory symptoms. Interleukin 1 blockade is a viable option for therapy.
authors.affiliation: Department of Dermatology, University of California, San Francisco, San Francisco, CA 94115, USA.
authors.forename: Hrishikesh
authors.initials: H
authors.lastname: Belani
authors.forename: Lianne
authors.initials: L
authors.lastname: Gensler
authors.forename: Urmila
authors.initials: U
authors.lastname: Bajpai
authors.forename: Eric
authors.initials: E
authors.lastname: Meinhardt
authors.forename: Jonathan
authors.initials: J
authors.lastname: Graf
authors.forename: Laura
authors.initials: L
authors.lastname: Pincus
authors.forename: Kieron S
authors.initials: KS
authors.lastname: Leslie
mesh_terms: D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007249:Inflammation / Q000097:blood / Q000150:complications* / Q000473:pathology; D007375:Interleukin-1 / Q000097:blood; D008875:Middle Aged; D009504:Neutrophils / Q000473:pathology*; D014581:Urticaria / Q000097:blood / Q000150:complications / Q000473:pathology*; D055815:Young Adult
chemical_list: D007375:Interleukin-1