Clinical experience and diagnostic algorithm of vulval Crohn’s disease

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Abstract

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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