**3.1 The NSAID-exacerbated cutaneous disease**

Patients with NECD suffer from spontaneous chronic urticaria and/or angioedema (CSU) and experience a worsening of these symptoms after NSAID intake. Most reactions appear between 1 to 12 hours after administering NSAID and last several days until the reaction is controlled with supplemental doses of antihistamines and/or corticosteroids. In up to 30 percent of cases facial, neck and hand angioedema may occur during the clinical exacerbation; an isolated angioedema is rarely a possible manifestation of NECD. Approximately 30% of patients with chronic spontaneous urticaria present exacerbations of their disease after NSAID exposure throughout their lives [1, 3–5, 8]. A positive correlation between the basal CSU activity disease and the possibility of a NSAID challenge reaction have been described in NECD patients [8].

