A reassessment of diagnostic criteria and treatment of idiopathic urticarial vasculitis: a retrospective study of 47 patients C. Tosoni, F. Lodi-Rizzini, M. Cinquini, G. Pasolini,* M. Venturini,* R. A. Sinico† and P. Calzavara-Pinton* Allergy Unit and *Department of Dermatology, Faculty of Medicine, Spedali Civili, Brescia, Italy; and †Department of Nephrology and Immunology, San Carlo Borromeo Hospital, Milan, Italy doi:10.1111/j.1365-2230.2008.02891.x Summary Background. Urticarial vasculitis (UV) is an uncommon type of chronic urticaria (CU), which exhibits leucocytoclastic vasculitis. Painful and long-lasting (> 24 h) weals associated with purpura or bruising are considered indicative of UV. It is often responsive to oral corticosteroids and poorly to oral antihistamines. Hypocomplementaemia and systemic involvement are also commonly reported. Aims. To diagnose patients with UV histologically and then compare their clinical features and response to various treatment regimens. Methods. Biopsies were taken from 312 subjects with CU unresponsive to oral antihistamines; of these, 47 were histologically diagnosed as having UV. Biopsies were taken irrespective of the clinical features of weal eruption. Other diseases known to be associated with small-vessel vasculitis had previously been excluded. Results. Individual weals lasted < 24 h in 57.4% of patients, and pain or tenderness was reported only by 8.6%. Extracutaneous features were present in 81%, hypocom- plementaemia in 11% and abnormalities of other laboratory parameters (i.e. raised erythrocyte sedimentation rate, microscopic haematuria) in 76.6%. Hydroxyzine was effective in only one patient. Both oral corticosteroids and cinnarizine were effective in a high percentage of the patients. Conclusion. This diagnostic approach allowed us to identify a large group (47 patients) with UV. Most did not present the clinical (prolonged duration of weals and bruising) and laboratory features that have previously been described as characteristic of UV. Cinnarizine was found to be a valuable treatment option. Introduction Urticarial vasculitis (UV) is believed to be an uncommon subset of chronic urticaria (CU) that is associated with leucocytoclastic vasculitis. 1–3 UV is most often idio- pathic, although it has been described in association with connective tissue diseases (systemic lupus erythe- matosus and Sjo ¨gren’s syndrome), neoplasia (mono- clonal gammopathy and lymphoma), chronic infection with hepatitis virus A, B and C, Epstein–Barr virus and Borrelia burgdorferi, drugs (cimetidine, fluoxetine, pro- cainamide, atenolol, sulfamethoxazole, paroxetine, sodium valproate, ciprofloxacin and zidovudine), and serum sickness-like diseases. 4 Idiopathic UV can be further subdivided into normo- complementaemic UV (NUV) and hypocomplement- aemic UV (HUV). NUV can be associated with systemic symptoms such as renal damage, arthralgia and other problems, but less often and with less severe damage than found with HUV. 4,5 Correspondence: Dr Piergiacomo Calzavara-Pinton, MD, Department of Dermatology, Azienda Ospedaliera Spedali Civili, P.le Spedali Civili 1, 25123 Brescia, Italy. E-mail: calzavar@med.unibs.it Conflict of interest: none declared. Accepted for publication 11 February 2008 Clinical dermatology • Original article Clinical and Experimental Dermatology Ó 2008 The Author(s) 166 Journal compilation Ó 2008 Blackwell Publishing Ltd • Clinical and Experimental Dermatology, 34, 166–170