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Nomenclature of Cutaneous Vasculitis: Dermatologic Addendum to the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides

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Sunderkötter CH1, Zelger B2, Chen KR3, Requena L4, Piette W5, Carlson JA6, Dutz J7, Lamprecht P8, Mahr A9, Aberer E10, Werth VP11, Wetter DA12, Kawana S13, Luqmani R14, Frances C15, Jorizzo J16, Watts JR17, Metze D18, Caproni M19, Alpsoy E20, Callen JP21, Fiorentino D22, Merkel PA23, Falk RJ24, Jennette JC24.


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Arthritis Rheumatol. 2018 Feb;70(2):171-184. doi: 10.1002/art.40375.

1 University Hospital of Halle, Halle (Saale), Germany, and University of Münster, Münster, Germany.

2 Medical University Innsbruck, Innsbruck, Austria.

3 Meguro Chen Dermatology Clinic, Tokyo, Japan.

4 Universidad Autónoma, Madrid, Spain.

5 John H. Stroger Jr. Hospital of Cook County and Rush University Medical Center, Chicago, Illinois.

6 Albany Medical College, Albany, New York.

7 University of British Columbia, Vancouver, British Columbia, Canada.

8 University of Lübeck, Lübeck, Germany.

9 Hospital Saint-Louis, University Paris 7, Paris, France.

10 Medical University of Graz, Graz, Austria.

11 University of Pennsylvania and Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, Pennsylvania.

12 Mayo Clinic, Rochester, Minnesota.

13 Nippon Medical School, Tokyo, Japan.

14 University of Oxford, Oxford, UK.

15 University Paris 6, Hôpital Tenon, Paris, France.

16 Wake Forest School of Medicine, Winston Salem, North Carolina.

17 Ipswich Hospital NHS Trust, Ipswich, UK, and Norwich Medical School, University of East Anglia, Norwich, UK.

18 University of Münster, Münster, Germany.

19 University of Florence, Florence, Italy.

20 Akdeniz University School of Medicine, Antalya, Turkey.

21 University of Louisville, Louisville, Kentucky.

22 Stanford University School of Medicine, Stanford, California.

23 University of Pennsylvania, Philadelphia.

24 University of North Carolina at Chapel Hill.


OBJECTIVE: To prepare a dermatologic addendum to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides (CHCC2012) to address vasculitides affecting the skin (D-CHCC). The goal was to standardize the names and definitions for cutaneous vasculitis.

METHODS: A nominal group technique with a facilitator was used to reach consensus on the D-CHCC nomenclature, using multiple face-to-face meetings, e-mail discussions, and teleconferences.

RESULTS: Standardized names, definitions, and descriptions were adopted for cutaneous components of systemic vasculitides (e.g., cutaneous IgA vasculitis as a component of systemic IgA vasculitis), skin-limited variants of systemic vasculitides (e.g., skin-limited IgA vasculitis, drug-induced skin-limited antineutrophil cytoplasmic antibody-associated vasculitis), and cutaneous single-organ vasculitides that have no systemic counterparts (e.g., nodular vasculitis). Cutaneous vasculitides that were not included in the CHCC2012 nomenclature were introduced.

CONCLUSION: Standardized names and definitions are a prerequisite for developing validated classification and diagnostic criteria for cutaneous vasculitis. Accurate identification of specifically defined variants of systemic and skin-limited vasculitides requires knowledgeable integration of data from clinical, laboratory, and pathologic studies. This proposed nomenclature of vasculitides affecting the skin, the D-CHCC, provides a standard framework both for clinicians and for investigators.