Letter to the Editor
Vulval Crohn's disease: A tertiary center experience of 23 patients
Article first published online: 28 APR 2011
Copyright © 2011 Crohn's & Colitis Foundation of America, Inc.
Inflammatory Bowel Diseases
Volume 17, Issue 7, page E77, July 2011
How to Cite
Landy, J., Peake, S.T.C., Akbar, A. and Hart, A.L. (2011), Vulval Crohn's disease: A tertiary center experience of 23 patients. Inflamm Bowel Dis, 17: E77. doi: 10.1002/ibd.21755
- Issue published online: 14 JUN 2011
- Article first published online: 28 APR 2011
To the Editor:
Crohn's disease (CD) affecting the vulva is often unrecognized and can be difficult to treat. The majority of cases reported describe metastatic CD, where the vulval inflammation is not contiguous with the gastrointestinal tract. Overall, only 56 cases of vulval CD are reported in the literature since the first description by Parks et al in 1965.1 We present our experience of 23 patients with vulval CD in a single tertiary center between 1999 and 2010. To our knowledge, this is the largest reported series of patients with vulval CD.
The inflammatory bowel disease (IBD) database was searched for all patients with a diagnosis of CD (based on standard clinical, endoscopic, and histological criteria), also affecting the vulva. Twenty-three patients were identified and their case notes were reviewed.
Five patients had metastatic vulval CD, while 18/23 had contiguous disease. Vulval involvement was the first presentation of CD for two patients. All of the patients were premenopausal and the median age at the time of vulval CD was 29 years (range, 19–50 years). The median interval from diagnosis of CD to vulval involvement was 10 years (range, 0–32 years).
The median age of CD diagnosis was 18 years (range, 13–40 years). Thirteen of the patients had previously undergone at least one surgical resection prior to their presentation of vulval disease. Seven had undergone proctocolectomy and four patients subtotal or hemicolectomy prior to presentation with vulval disease. Seventeen patients had perianal disease. Fifteen patients had colonic disease distribution, 7 ileocolonic, and 1 patient had ileocolonic and upper gastrointestinal disease. Five patients had extraintestinal manifestations (four arthropathy, one erythema nodosum). Eight patients were smokers.
Five patients underwent biopsy to confirm the diagnosis of vulval CD. Histology demonstrated mixed inflammatory cell infiltrates with noncaseating epithelioid granuloma formation. Stains for fungi and acid-fast bacilli were negative. Presenting symptoms varied, with a wide range of symptoms. The most frequent presentations were labial swelling (12 patients), erythema (10 patients), and pain (nine patients). Other presentations included discharge, abscesses, ulceration, and pruritus.
Twelve patients were initially treated with antibiotic therapy, most commonly ciprofloxacin and metronidazole (orally and topically). Three patients were treated with topical steroids and three patients with topical tacrolimus. Ten patients were treated with biological therapy, nine initially with infliximab, and one initially with adalimumab. Sixteen of the patients were already treated with an immunosuppressive agent at the time of diagnosis of vulval CD (13 azathioprine or 6-mercaptopurine; three methotrexate). Two patients required incision and drainage of vulval abscesses and one patient underwent vulvectomy.
Patients receiving topical steroids showed an initial response with an improvement of vulval symptoms, but in all cases response was not sustained. Two of the three patients treated with topical tacrolimus showed a rapid improvement in symptoms within 1 week of initiating treatment, but this improvement was not sustained for more than 2 months. Of the 10 patients treated with biological therapy, one patient had an infusion reaction and three did not respond. For five patients vulval symptoms were improved after an induction course of infliximab. The patient initially treated with adalimumab demonstrated improvement of vulval symptoms within 3 doses (160/80/40 mg). There was a loss of response to infliximab in four of the five primary responders. One regained response with increased frequency of infliximab; two patients were switched to adalimumab with good response.
An awareness of vulval CD is necessary to identify those with vulval symptoms due to contiguous disease as well as metastatic disease. The majority of patients are young and have had a severe course of disease, often with previous surgery. Presentation is varied, but labial swelling, pain, and erythema are the most frequent presentations. Surgery is rarely required and patients may gain short-term improvement with antibiotics, topical steroids, or topical tacrolimus. Infliximab or adalimumab can be of benefit in the long-term treatment of patients with vulval CD.
- 1Crohn's disease with cutaneous involvement. Proc R Soc Med. 1965; 58: 241–242., , .
J. Landy MRCP*, S.T.C. Peake MRCP*, A. Akbar MRCP, PhD*, A.L. Hart MRCP, PhD*, * Department of Gastroenterology St Mark's Hospital, Harrow London, UK.