Secondary Logo

Journal Logo

Institutional members access full text with Ovid®

An update on the nomenclature for cutaneous vasculitis

Caproni, Marziaa; Verdelli, Aliceb

Current Opinion in Rheumatology: January 2019 - Volume 31 - Issue 1 - p 46–52
doi: 10.1097/BOR.0000000000000563
VASCULITIS SYNDROMES: Edited by Hasan Yazici and Yusuf Yazici

Purpose of review Cutaneous vasculitis reflects a spectrum ranging from skin limited to severe systemic forms. To date, there is still no generally acknowledged nomenclature for cutaneous vasculitis. This review aims to summarize the recent advances in the nomenclature of cutaneous vasculitis.

Recent findings The most widely adopted vasculitis classification system is the one of 2012 Revised Chapel Hill Consensus Conference (CHCC) which represent not such a classification but a nomenclature system that name vasculitis on the basis of the size of the vessel affected. The CHCC 2012 did not deal with the special features of cutaneous vasculitis and did not explicitly discuss the presence of skin-limited or skin-dominant forms of vasculitis. Therefore, a consensus group was formed to propose an Addendum to CHCC 2012, focusing on cutaneous vasculitis. The Addendum better clarify the main aspects of some single-organ vasculitis, including IgM/IgG vasculitis, nodular vasculitis, erythema elevatum et diutinum and recurrent macular vasculitis in hypergammaglobulinemia. Moreover, it differentiated normocomplementemic from hypocomplementemic urticarial vasculitis. Finally, it recognized cutaneous polyarteritis nodosa as a distinct subtype of polyarteritis nodosa.

Summary Classification criteria are useful tools to standardize names and definitions for cutaneous vasculitis; however, they do not represent diagnostic criteria. Collaborative efforts are still needed to get a shared classification and valid diagnostic criteria for cutaneous vasculitis.

aU.O. Dermatology I, P.O. Piero Palagi, USL Toscana Centro

bSection of Dermatology, Department of Surgery and Translational Medicine, University of Florence, Florence, Italy

Correspondence to Marzia Caproni, U.O. Dermatology I, P.O. Piero Palagi, USL Toscana Centro, University of Florence, Viale Michelangiolo, 41, 50125 Florence, Italy. Tel: +39 055 6939627; fax: +39 055 468211; e-mail:

Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.