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Int J Clin Exp Pathol 2013;6(10):2263-2266

Case Report
Metastatic Crohn’s disease accompanying granulomatous vasculitis and lymphangitis in
the vulva

Mitsuaki Ishida, Muneo Iwai, Keiko Yoshida, Akiko Kagotani, Hidetoshi Okabe

Department of Clinical Laboratory Medicine and Division of Diagnostic Pathology, Shiga University of Medical Science, Shiga, Japan

Received July 22, 2013; Accepted August 15, 2013; Epub September 15, 2013; Published October 1, 2013

Abstract: 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. (IJCEP1307039).

Keywords: Metastatic Crohn’s disease, granulomatous vasculitis, granulomatous lymphangitis, vulva

Address correspondence to: Dr. Mitsuaki Ishida, Department of Clinical Laboratory Medicine and Division of Diagnostic Pathology, Shiga
University of Medical Science, Tsukinowa-cho, Seta, Otsu, Shiga, 520-2192, Japan. Tel: +81-77-548-2603; Fax: +81-77-548-2407; E-mail:
mitsuaki@belle.shiga-med.ac.jp