Ishida Mitsuaki, Iwai Muneo, Yoshida Keiko, Kagotani Akiko, Okabe Hidetoshi
Department of Clinical Laboratory Medicine and Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.
Int J Clin Exp Pathol. 2013 Sep 15;6(10):2263-6. eCollection 2013.
Metastatic Crohn's disease (CD) is an extremely rare extragastrointestinal manifestation of CD, and is characterized histopathologically by the presence of non-caseating granulomatous inflammation. Granulomatous vasculitis and lymphangitis have rarely been documented in metastatic CD. Herein, we report the first documented case of metastatic CD accompanied by both granulomatous vasculitis and lymphangitis in the vulva. A 35-year-old Japanese female with CD presented with multiple small nodules in her vulva. Biopsy was performed under a clinical diagnosis of genital warts. A histopathological study revealed marked lymphangiectasia in the papillary dermis. Within the dilated lymphatics, lymphocytes and aggregates of macrophages were present, which are typical features of granulomatous lymphangitis. Tiny non-caseating granulomas and granulomatous vasculitis were also observed. Accordingly, a diagnosis of metastatic CD accompanied by both granulomatous vasculitis and lymphangitis was made. The occurrence of cutaneous lesions in patients with CD is well known. Albeit extremely rare, lymphangiectasia has been reported in the vulva of CD patients that clinically mimicked viral warts, as in the present case. The diagnosis of metastatic CD in the present case was not difficult because characteristic histopathological features were present, and a clinical history of CD was available. However, a few cases of genital swelling associated with granulomatous inflammation prior to a diagnosis of gastrointestinal CD have been documented. Therefore, granulomatous vasculitis and lymphangitis in the external genitals should be considered as potential indication of metastatic CD even in cases without a history of gastrointestinal CD.
转移性克罗恩病（CD）是CD极为罕见的胃肠道外表现，其组织病理学特征为非干酪样肉芽肿性炎症。肉芽肿性血管炎和淋巴管炎在转移性CD中鲜有报道。在此，我们报告首例有文献记载的外阴部转移性CD伴肉芽肿性血管炎和淋巴管炎的病例。一名35岁患CD的日本女性，外阴出现多个小结节。在临床诊断为尖锐湿疣的情况下进行了活检。组织病理学研究显示乳头真皮层有明显的淋巴管扩张。在扩张的淋巴管内，可见淋巴细胞和巨噬细胞聚集，这是肉芽肿性淋巴管炎的典型特征。还观察到微小的非干酪样肉芽肿和肉芽肿性血管炎。因此，诊断为转移性CD伴肉芽肿性血管炎和淋巴管炎。CD患者出现皮肤病变是众所周知的。尽管极为罕见，但如本病例一样，CD患者外阴部出现临床上类似病毒疣的淋巴管扩张已有报道。本病例中转移性CD的诊断并不困难，因为存在特征性的组织病理学特征且有CD的临床病史。然而，已有一些在诊断胃肠道CD之前出现与肉芽肿性炎症相关的生殖器肿胀的病例记录。因此，即使在没有胃肠道CD病史的病例中，外生殖器的肉芽肿性血管炎和淋巴管炎也应被视为转移性CD的潜在指征。
