Azuma Naoto, Natsuaki Masaru, Yamanishi Kiyofumi, Kondo Nobuyuki, Iwasaki Tsuyoshi, Morimoto Mai, Nishioka Aki, Sekiguchi Masahiro, Kitano Masayasu, Hashimoto Naoaki, Matsui Kiyoshi, Sano Hajime
Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan.
Nihon Rinsho Meneki Gakkai Kaishi. 2010;33(3):149-53. doi: 10.2177/jsci.33.149.
A 25-year-old-man was referred with an approximately 1-year history of recurrent fever, tonsillitis, testicular pain and testicular swelling in June 2009. He also complained of visual disturbance, some erythema nodosum (EN) like lesions on his lower extremities and oral aphthous ulcerations for 5 months. Opthalmological consultation confirmed retinochoroiditis, and ultrasonography revealed epididymitis. A biopsy of the EN like lesion showed a necrotizing vasculitis of the small and medium-sized vessels with septal panniculitis in the subcutis. The laboratory findings revealed an elevation of CRP and positive HLA-B51. He was diagnosed with incomplete Behcet's disease according to the Japanese criteria (1987). After the initiation of the therapy with 10 mg/day of prednisolone, his symptoms promptly ameliorated. In our case, although epididymitis as well as the histopathological findings suggested polyarteritis nodosa (PN), a diagnosis of BD was established on the basis of other contemporaneous findings. Necrotizing vasculitis as a cutaneous manifestation in patients with BD has been rarely reported. However, BD and PN, including cutaneous PN, have rarely described in conjunction. Therefore, we propose that PN-like necrotizing vasculitis might be the subtype of BD.
一名25岁男性于2009年6月因反复发热、扁桃体炎、睾丸疼痛及睾丸肿胀约1年前来就诊。他还诉说有5个月的视力障碍、下肢一些结节性红斑样损害及口腔溃疡。眼科会诊确诊为视网膜脉络膜炎，超声检查显示附睾炎。结节性红斑样损害的活检显示中小血管坏死性血管炎，皮下有间隔性脂膜炎。实验室检查结果显示CRP升高且HLA - B51阳性。根据日本标准（1987年），他被诊断为不完全型白塞病。开始使用10毫克/天泼尼松龙治疗后，他的症状迅速改善。在我们的病例中，尽管附睾炎以及组织病理学表现提示结节性多动脉炎（PN），但基于其他同时出现的表现确诊为白塞病。白塞病患者中作为皮肤表现的坏死性血管炎鲜有报道。然而，白塞病与PN，包括皮肤型PN，很少同时被描述。因此，我们提出PN样坏死性血管炎可能是白塞病的一个亚型。
