Single-organ cutaneous small-vessel vasculitis according to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides: a study of 60 patients from a series of 766 cutaneous vasculitis cases

• Published in: Rheumatology (Oxford, England) Vol. 54; no. 1; pp. 77 - 82
• Main Authors: Loricera, Javier, Blanco, Ricardo, Ortiz-Sanjuán, Francisco, Hernández, José L, Pina, Trinitario, González-Vela, M Carmen, Calvo-Río, Vanesa, Rueda-Gotor, Javier, Alvarez, Lino, González-López, Marcos A, Marcellán, María, González-Gay, Miguel A
• Format: Journal Article
• Language: English
• Published: England 01-01-2015
• Subjects: Adrenal Cortex Hormones - therapeutic use; Adult; Aged; Anti-Inflammatory Agents, Non-Steroidal - therapeutic use; Female; Follow-Up Studies; Humans; Male; Middle Aged; Retrospective Studies; Skin - blood supply; Terminology as Topic; Treatment Outcome; Vasculitis, Leukocytoclastic, Cutaneous - classification; Vasculitis, Leukocytoclastic, Cutaneous - diagnosis; Vasculitis, Leukocytoclastic, Cutaneous - drug therapy; Chapel Hill Consensus Conference; cutaneous leucocytoclastic angiitis; single cutaneous small vessel vasculitis; single-organ vasculitis; cutaneous vasculitis
• ISSN: 1462-0324; 1462-0332
• DOI: 10.1093/rheumatology/keu295

Cutaneous vasculitis (CV) encompasses a wide group of entities characterized by inflammation of skin blood vessels. The term single-organ vasculitis was recently coined by the 2012 Chapel Hill Consensus Conference (CHCC) to define vasculitis affecting a single organ. To our knowledge there are no published reports on single-organ cutaneous small vessel vasculitis (SoCSVV). Our aim was to characterize this entity from a wide series of patients with CV.
We analysed cases of SoCSVV from a series of 766 patients with CV from a single university referral centre. According to 2012 CHCC, the following conditions were required to define SoCSVV: (i) skin biopsy showing characteristic leucocytoclastic vasculitis and (ii) vasculitis limited to skin.
We included 60 patients (26 women and 34 men) with a mean age of 56 years. The main precipitating factors for SoCSVV were drugs [26 patients (52%)] and previous infection [17 patients (34%)]. The main clinical manifestations were palpable purpura (81.7%) and fever (18.3%). The most frequent laboratory findings were leucocytosis and elevated ESR. Nearly one-quarter of patients with SoCSVV required pharmacological therapy. Corticosteroids (15%) and NSAIDs (13.3%) were the main agents prescribed. After a median follow-up of 4 months, complete recovery was observed in all the patients, although relapses occurred in 8% of patients.
SoCSVV defined according to the 2012 CHCC may be considered a benign disease usually associated with drugs and/or a previous infection.