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. 2020 Jan 10;11:179. doi: 10.1038/s41467-019-13984-8

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© The Author(s) 2020

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

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Fig. 4 — a Representative image from serial sections of the lesional skin of FACAS patient ca. 1 h after cold exposure. FXII immunoreactivity is restricted to neutrophils at perivascular sites and within blood vessels (white arrows), n = 2 technical replicates. b Exemplary images from cytospins isolated from the peripheral blood of FACAS patients and healthy control subjects. Only single FACAS neutrophils (0.72%), but not healthy control neutrophils (0%), present with double-positive cytoplasmatic staining for FXII and MPO. Original magnification ×400, n = 3 technical replicates. c Peripheral blood mononuclear cells were isolated from healthy control subjects by density-gradient centrifugation and stimulated with increasing doses of bradykinin. IL-8 secretion was assessed by ELISA. Pooled data of n = 3 different donors, n = 2 technical replicates. The graph shows individual data points and the mean ± s.e.m. Statistical significance is indicated by one-way ANOVA with Sidak’s multiple comparisons. Source data are provided as a source data file.