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Cutaneous arteritis with intimal fibrin ring and immature myeloid cell infiltrate: lymphocytic thrombophilic arteritis or histiocytoid polyarteritis nodosa?

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Abstract

The ongoing debate on whether lymphocytic thrombophilic arteritis (LTA) is a separate disease or a type of polyarteritis nodosa (PAN) has yet to be settled. In this study, we analyzed the nature of infiltrating cells in LTA to resolve this controversy. Skin biopsies from five female patients (mean age 29.4 years, age range 16–45 years) diagnosed with LTA were immunostained for CD3, CD20, CD68, lysozyme, myeloid cell nuclear differentiation antigen, myeloperoxidase, and PU.1. Immunohistochemistry revealed that the majority of mononuclear cells in all five cases were not lymphocytes but myelomonocytic cells. Given that the infiltrating cells are of the myelomonocyte lineage including immature myeloid cells, PAN was deemed the more appropriate diagnosis for the five cases rather than LTA. Whether PAN with immature myeloid cells (histiocytoid PAN) is the same disease as conventional PAN with mature neutrophils requires further investigation.

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Author notes

  1. Masakazu Fujimoto, Toshiya Miyake and Yo Kaku contributed equally to this work.

Authors and Affiliations

  1. Department of Diagnostic Pathology, Kyoto University Hospital, 54 Shogoin Kawahara-Cho, Sakyo-Ku, Kyoto, 606-8507, Japan

    Masakazu Fujimoto, Masahiro Hirata & Hironori Haga

  2. Department of Dermatology, Kyoto University Graduate School of Medicine, 54 Shogoin Kawahara-Cho, Sakyo-Ku, Kyoto, 606-8507, Japan

    Toshiya Miyake, Yo Kaku & Kenji Kabashima

Authors
  1. Masakazu Fujimoto
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  2. Toshiya Miyake
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  3. Yo Kaku
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  4. Masahiro Hirata
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  5. Kenji Kabashima
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  6. Hironori Haga
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Contributions

MF, TM, and YK drafted the article. MF and YK analyzed the pathological data. TM and YK acquired and analyzed the clinical data. MH performed immunostainings. All authors approved the final version of this manuscript for publication.

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Correspondence to Masakazu Fujimoto or Yo Kaku.

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Fujimoto, M., Miyake, T., Kaku, Y. et al. Cutaneous arteritis with intimal fibrin ring and immature myeloid cell infiltrate: lymphocytic thrombophilic arteritis or histiocytoid polyarteritis nodosa?. Virchows Arch 482, 1079–1083 (2023). https://doi.org/10.1007/s00428-023-03531-8

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  • DOI: https://doi.org/10.1007/s00428-023-03531-8

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