title: 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.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  SoCSVV defined according to the 2012 CHCC may be considered a benign disease usually associated with drugs and/or a previous infection.
authors: Departments of Rheumatology, Internal Medicine, Pathology, Pediatrics and Dermatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain. miguelaggay@hotmail.com.
authors: Javier
authors: J
authors: Loricera
authors: Departments of Rheumatology, Internal Medicine, Pathology, Pediatrics and Dermatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
authors: Ricardo
authors: R
authors: Blanco
authors: Departments of Rheumatology, Internal Medicine, Pathology, Pediatrics and Dermatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
authors: Francisco
authors: F
authors: Ortiz-Sanjuán
authors: Departments of Rheumatology, Internal Medicine, Pathology, Pediatrics and Dermatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
authors: José L
authors: JL
authors: Hernández
authors: Departments of Rheumatology, Internal Medicine, Pathology, Pediatrics and Dermatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
authors: Trinitario
authors: T
authors: Pina
authors: Departments of Rheumatology, Internal Medicine, Pathology, Pediatrics and Dermatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
authors: M Carmen
authors: MC
authors: González-Vela
authors: Departments of Rheumatology, Internal Medicine, Pathology, Pediatrics and Dermatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
authors: Vanesa
authors: V
authors: Calvo-Río
authors: Departments of Rheumatology, Internal Medicine, Pathology, Pediatrics and Dermatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
authors: Javier
authors: J
authors: Rueda-Gotor
authors: Departments of Rheumatology, Internal Medicine, Pathology, Pediatrics and Dermatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
authors: Lino
authors: L
authors: Alvarez
authors: Departments of Rheumatology, Internal Medicine, Pathology, Pediatrics and Dermatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
authors: Marcos A
authors: MA
authors: González-López
authors: Departments of Rheumatology, Internal Medicine, Pathology, Pediatrics and Dermatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
authors: María
authors: M
authors: Marcellán
authors: Departments of Rheumatology, Internal Medicine, Pathology, Pediatrics and Dermatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain. miguelaggay@hotmail.com.
authors: Miguel A
authors: MA
authors: González-Gay
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D005500:Follow-Up Studies; D012189:Retrospective Studies; D012867:Skin / Q000098:blood supply; D009626:Terminology as Topic; D016896:Treatment Outcome; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy; D003245:Consensus Statements as Topic
chemical_list: D000305:Adrenal Cortex Hormones; D000894:Anti-Inflammatory Agents, Non-Steroidal
keywords: Chapel Hill Consensus Conference; cutaneous leucocytoclastic angiitis; cutaneous vasculitis; single cutaneous small vessel vasculitis; single-organ vasculitis