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Table 1 Summary of the differential diagnosis of urticaria, arthritis and fever from the literature

From: Acute adult-onset still’s disease presenting as pulmonary hemorrhage, urticaria, angioedema and leukemoid reaction: a case report and literature review

Classes of diseases Diseases
Hereditary auto-inflammatory syndromes Familial cold urticaria
  Cryopyrin-associated syndrome
  Muckle–Wells syndrome
  Familial cold urticaria
  Chronic infantile neurologic cutaneous and
  articular syndrome (CINCA/NOMID)
Infectious diseases Hepatitis B, C
  Chronic meningococcemia
  STAR complex (Sore Throat, Arthritis and Rash secondary to a viral infection)
  Streptococcal pharyngitis with reactive arthralgia and exanthem
Autoimmune disorders AOSD
  Systemic lupus erythematosus
  Acquired C1 esterase deficiency
  Schnitzler syndrome
Hematological disorders Lymphoma
  Monoclonal gammopathy of unknown significance
  Multiple myeloma
  Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndromeº
  Waldenström’s macroglobulinemia
Other Hypocomplementic urticarial vasculitis
  Idiopathic chronic urticaria
  Delayed pressure urticaria
  Cryoglobulinemia
  Behçet’s disease
  Mastocytosis
  1. Modificated and update from: (Affleck and Littlewood 2005; Koning et al. 2007; Kyle and Rajkumar 2003; Csepregi and Nemesánszky 2000; Davis and Brewer 2004; Hawkins et al. 2004; Tomkova et al. 1998; Zuberbier et al. 2009; Deacock 2008).