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Clinical experience and diagnostic algorithm of vulval Crohn's disease
Lotte Boxhoorn1, Tom J Stoof, Tim de Meij
1Departments of aGastroenterology and Hepatology bGynaecology cPediatric Gastroenterology, VU University Medical Center dDepartment of Gastroenterology and Hepatology, Academic Medical Center, Amsterdam eDepartment of Gastroenterology and Hepatology, Radboud University Medical Center, Nijmegen fDepartment of Gastroenterology and Hepatology, University Medical Center, Utrecht gDepartment of Gastroenterology, Geriatrics, Internal and Intensive Care Medicine, Zuyderland Medical Center, Heerlen-Sittard-Geleen, The Netherlands.
Background And Aim:
Vulval Crohn's disease (VCD) is a rare extraintestinal cutaneous manifestation of Crohn's disease. No consensus on the diagnostic workup and therapeutic management of this condition has been provided in the current literature.
Patients And Methods:
Retrospective, multicentre descriptive case series of female patients diagnosed and treated with VCD. By chart review, data on initial symptoms, clinical courses, histologic findings and therapeutic management were collected.
Results:
Fifteen female patients with a median age of 28 years (interquartile range: 28-44 years) suffering from Crohn's disease of the ileum (27%), colon (33%) and ileocolon (40%) were included. VCD manifested most frequently with vulval swelling (93%), pain (80%) and erythema (73%). Histologic analysis demonstrated granulomatous inflammation in 78% and a mixed inflammatory cell infiltrate in 67% of cases. In eight (53%) cases, topical therapy resulted in temporary reduction of vulval symptoms. Combotreatment with immunosuppressive agents and tumour necrosis factor α inhibitors was the most effective second-line therapy: five (33%) patients achieved sustained clinical remission with this therapeutic strategy.
Conclusion:
The diagnostic workup of VCD is challenging and should be approached in a multidisciplinary manner. Histopathologic analysis of the vulva supports the diagnosis. Topical therapy and systemic treatment with immunosuppressive agents and tumour necrosis factor α inhibitors are advised to treat this condition.
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