Cutaneous polyarteritis nodosa: revisiting its definition and diagnostic criteria
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Polyarteritis nodosa (PN) is a classical collagen disease with poor prognosis that demonstrates systemic necrotizing vasculitis of small and medium-sized arteries. Cutaneous symptoms are observed in 25–60% of PN patients. On other hand, cutaneous polyarteritis nodosa (CPN) is designated for the cutaneous limited form of PN and demonstrates benign prognosis. However, there has been much debate on whether or not CPN can progress to PN. Although CPN lesions are fundamentally limited to skin, some CPN cases show extracutaneous symptoms such as peripheral neuropathy and myalgia. According to PN diagnostic criteria, which were established by the Ministry of Health, Labour and Welfare of Japan, a disease with both cutaneous and at least one extracutaneous symptom with appropriate histopathological findings can be diagnosed as PN. The same is true according to diagnostic criteria established by the American College of Rheumatology. In addition, there are no specific diagnostic criteria for CPN. In this study, CPN cases were retrospectively collected from multiple Japanese clinics, and analyzed for detailed clinical and histopathological manifestations, in order to redefine the clinical entity of CPN and to propose appropriate diagnostic criteria for CPN and PN. According to the CPN description in Rook’s Textbook of Dermatology, we collected 22 cases with appropriate histopathological findings. Of the 22 cases, none progressed to PN or death during the follow-up period, 32% had peripheral neuropathy and 27% had myalgia. Regarding extracutaneous symptoms with CPN, 17 dermatological specialists in vasculitis sustained the opinion that CPN can be accompanied by peripheral neuropathy and myalgia but these symptoms are limited to the same area as skin lesions. Based on these results, we devised new drafts for CPN and PN diagnostic criteria. Our study shows the efficacy of these criteria and most dermatologists recognized that our new diagnostic criteria for CPN and PN are appropriate at the present time. In conclusion, this study suggests that CPN does not progress to PN, and introduces new drafts for CPN and PN diagnostic criteria.
KeywordsCutaneous polyarteritis nodosa Clinical entity Definition Diagnostic criteria Extracutaneous symptoms
We are grateful to the following dermatologists for their cooperation in providing information on CPN patients and providing answer to our questionnaires; Hiroyuki Okamoto, MD (Kansai Medical University), Atsushi Utani, MD (Kyoto University), Yayoi Nagai, MD (Gunma University), Osamu Yamasaki, MD (Okayama University), Taisuke Itoh, MD (Hamamatsu University School of Medicine), Takatoshi Shimauchi, MD (University of Occupational and Environmental Health), Toshiyuki Yamamoto, MD*, Yumiko Furuhata, MD (Tokyo Medical University), Ko-Ron Chen, MD (Saiseikai Central Hospital), Tamihiro Kawakami, MD (St. Marianna University School of Medicine), Naoko Ishiguro, MD (Tokyo Women’s Medical University), Setsuya Aiba, MD (Tohoku University), Kensei Katsuoka, MD (Kitasato University), Mikio Masuzawa, MD (Kitasato University), Seiji Kawana, MD (Nippon Medical School), Shinichi Sato, MD (Nagasaki University), Eishin Morita, MD (Shimane University), Seiichi Izaki, MD (Saitama Medical University). * Currently at the Department of Fukushima Medical University.
Conflict of interest statement
The authors have no potential conflict of interest.
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