Metastatic Vulval Crohn's Disease

Published Date: 19th July 2016

Publication Authors: Idama TO, Myagerimath R

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Background 

Crohn’s disease is a chronic granulomatous, inflammatory disorder and may have intestinal/extra intestinal manifestations. Vulval involvement is an uncommon extra intestinal manifestation of Crohn’s disease. It is not uncommon to encounter diagnostic dilemmas in vulval lesions. We present a case of abnormal vulval lesions due to Crohn’s disease.

Case 

A 42-year-old woman was referred with a history of recurrent vulval lesions; which started on the right side but was eventually noted on both the sides. She was treated with antibiotics on several occasions with a suspicion of infection of Bartholin’s gland with no relief. Twenty years ago she had right hemicolectomy for Crohn’s disease of the ileum and had remained asymptomatic. On examination a 2 9 3 cm indurated area was noted on either side of the lower 2/3rd of vulva with two sinus tracks on the top. These areas were excised and sent for histology, which confirmed nonspecific inflammation with abscesses, in addition to epitheliod granulomas suggestive of Crohn’s disease of the vulva. Postoperatively she was on long term metronidazole for 6 months; skin lesions healed eventually.

Discussion 

Vulval involvement of Crohn’s disease can appear before or after intestinal problems or it may occur simultaneously. It may be by virtue of contiguity, as a direct extension of intestinal involvement, or non-contiguous (metastatic) in which there is no connection between the vulva and the bowel. The metastatic lesions follow a course independent of the digestive disease raising therapeutic problems in their localisation. In a review by Andreani et al., 91% of cases of vulvar Crohn’s disease had metastatic spread, while only 5% had contiguous spread. In the same study 25% of vulvar Crohn’s disease did not have any intestinal involvement at the time of the vulvar lesion; as noted in our index case. It is in these cases that making a correct diagnosis becomes difficult; thorough clinical evaluation and biopsy are needed for proper diagnosis and management. Recent advances in medical management of Crohn’s disease have reduced surgical interventions. But advanced cases of vulval Crohn’s disease may require vulvectomy, as local excision has been reported to show recurrence of the disease.

Conclusion 

Vulval Crohn’s disease is rare, can manifest without intestinal symptoms and should be considered as a differential diagnosis of vulval lesions at any age. Multidisciplinary approach is paramount and early biopsy is advisable to avoid delay in diagnosis.

Myagerimath, R and Idama, T. (2013).  Metastatic Vulval crohn's disease, a rare entity . BJOG: An International Journal of Obstetrics and Gynaecology. 120 (Supplement S1), 505-06

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