Metastatic Vulval Crohn’s Disease With Good Outcome on Ustekinumab

Abstract

Vulval Crohn's disease is a rare manifestation of Crohn's disease. Although it is usually caused by a fistulating process, it may be a result of a metastatic disease process as well, the exact mechanisms of which are not well understood yet. Vulval Crohn's disease may occur before the onset of bowel symptoms and may continue to be active while bowel symptoms are quiescent. Coupled with the fact that most vulval Crohn's lesions are similar to other disease processes, it can prove to be a diagnostic challenge. Due to its rarity, no guidelines for treatment exist; however, most reports agree that when symptoms are not controlled with antibiotics and topical steroids, immunosuppressive medications such as oral steroids and biological agents are the way forward. Although surgery may be an option, data suggest that its use is scarce. We report the case of a 47-year-old Caucasian woman with bilateral metastatic vulval Crohn's disease from a district hospital in the United Kingdom who responded well to ustekinumab. This is one of the first few cases reporting good clinical outcomes with the agent.

Successful multidisciplinary treatment of the rare pathology of vulvar Crohn’s disease

“…If the initial medical therapy should fail then escalation to biologics such as anti-Tumor necrosis factor (TNF)-a inhibitors has shown a favourable response in multiple case reports 14. One case report documented a favourable response with ustekinumab which can be used if anti-TNF-a inhibitors should fail, are contraindicated or not tolerated 15. Immunomodulators (IMM) such as azathioprine should be used alongside anti-TNF-a inhibitors not only for direct treatment of the pathology but also in the prevention of the formation of antidrug antibodies (ADA) against the anti-TNF-a inhibitor which could render it ineffective.…”

Use of ustekinumab in vulvovaginal lesions associated with Crohn's disease (clinical case)

“…(2020) и Y. Lim, M. Singh (2021) в своих публикациях делятся впечатлением о скорости развития положительного эффекта и быстром исчезновении симптомов генитального поражения МетБК после начала терапии устекинумабом [6,27].…”

“…МетБК была впервые описана Parks et al в 1965 году [4] и определяется как возникновение гранулематозных поражений, характерных для БК, на участке кожи в любом месте, удаленном от желудочно-кишечного тракта. При МетБК могут поражаться гениталии, что относят к редким кожным внекишечным проявлениям БК [5,6]. Хотя большинство поражений гениталий может быть вызвано свищевым процессом, возможно развитие МетБК гениталий без возникновения свищей, фистул и изъязвлений на половых органах и в очень редких случаях вследствие распространения гангренозной пиодермии [6,7].…”

“…При МетБК могут поражаться гениталии, что относят к редким кожным внекишечным проявлениям БК [5,6]. Хотя большинство поражений гениталий может быть вызвано свищевым процессом, возможно развитие МетБК гениталий без возникновения свищей, фистул и изъязвлений на половых органах и в очень редких случаях вследствие распространения гангренозной пиодермии [6,7]. Следует отметить, что поражение гениталий может предшествовать, совпадать или развиваться после диагностики БК [8].…”

Gastrointestinal: A rare case of concurrent vulvar Crohn's disease and pyoderma gangrenosum

“…Crohn's disease (CD) is a chronic inflammatory condition affecting the gastrointestinal tract that can result in extraintestinal inflammation 1,2 . Involvement of the vulva is a rare extraintestinal manifestation of CD 3 with one study reporting vulvar CD affecting less than 0.25% of women with inflammatory bowel disease (IBD). Pyoderma gangrenosum (PG) is an autoinflammatory dermatitis with neutrophilic dysfunction, which has also been infrequently seen in patients with IBD 4,5 .…”