Recommendations for the diagnostic work‐up of cutaneous small vessel vasculitis – Position Statement of the European Academy of Dermatology and Venereology Vasculitis and Vasculopathy Task Force

作者：Erkan Alpsoy, Marzia Caproni, David A. Wetter, Matthias Goebeler, N. J. Levell, Stefan W. Schneider, Carmen Sălăvăstru, Mar Llamas‐Velasco, Angelo Valerio Marzano, Melih Akyol, Jan Dutz, M. Jachiet, Tamihiro Kawakami, Elena Biancamaria Mariotti, Carlo Alberto Maronese, Robert G. Micheletti, J.‐B. Monfort, Warren W. Piette, Gudrun Ratzinger, Jacek C. Szepietowski, Victoria P. Werth, Cord Sunderkötter · 发表于：Journal of the European Academy of Dermatology and Venereology · 年份：2025 · DOI：10.1111/jdv.70249 · 被引用次数：2 · 研究领域：Vasculitis and related conditions、Systemic Sclerosis and Related Diseases、Otitis Media and Relapsing Polychondritis

BACKGROUND: Small vessel vasculitides (SVV) comprise a heterogeneous group of cutaneous SVV (CSVV) that can be skin-limited or systemic. Given their diverse forms and symptoms and the lack of a standardized diagnostic approach, it is crucial to establish clear and consensus-driven recommendations for diagnosis. OBJECTIVES: To standardize diagnosis for the detection of the type, extent, potential causes or underlying conditions of CSVV. METHODS: International experts comprised the panel. The Delphi panel data was collected through two rounds of questionnaires. Agreement was measured using an 11-point Likert scale (0 = strongly disagree, 10 = strongly agree). A 7-vote minimum was required for agreement on each statement. Delphi panel statements scoring 3-7 were discussed during the meeting. RESULTS: 20 experts participated in the first round of the Delphi panel, while 17 experts took part in the second. They agreed that the diagnosis of CSVV relies on a combination of clinical manifestations, laboratory findings and histopathology. Palpable purpura on the lower extremities is recognized as the most reliable hallmark for most SVV and a sign of immune complex vasculitis. When the latter is suspected, a skin biopsy for direct immunofluorescence (DIF) should be obtained from an early, partially blanchable macule because the type of perivascular immunoglobulin is important for further diagnosis. The proper selection of biopsy sites for routine HE biopsy depends on the morphology of ...