Vulvar swelling as the first presentation of Crohn's disease in children—A report of three cases

Abstract

Vulvar swelling is a rare manifestation of metastatic Crohn's disease in children. It often predates gastrointestinal symptoms and can be the first presentation of pediatric Crohn's disease. We report three cases of pediatric Crohn's disease presenting with vulvar swelling. We discuss this rare presentation and its treatment and highlight the importance of recognizing it so that prompt investigation and appropriate management can be initiated.

Lymphatic drainage abnormalities in a child presenting as an unilateral vulvar swelling: a case of ‘Bicyclist's vulva’

“…Indeed, an isolated vulvar swelling in paediatric patients must eliminate several diagnoses. First, vulvar swelling could be a rare manifestation of metastatic Crohn's disease in children, 5 but it was poorly constituent here, with the absence of noncaseating granulomas in skin biopsy. Then, the childhood asymmetry labium majus enlargement, an uncommon benign condition that occurs in prepubertal girls, was excluded because of the lack of pain and the lymphatic anomalies found on the lymphoscintigraphy.…”

Vulvar Crohn’s disease in an adolescent diagnosed after unsuccessful surgical treatment

“…These cases of VCD are usually accompanied by gastrointestinal fistulas, but most do not undergo gastrointestinal evaluation when they do not present with recognizable gastrointestinal symptoms [ 18 – 20 ]. They are usually diagnosed at adulthood, with a mean age at diagnosis of 34 years [ 4 ], and only a few cases have been diagnosed in children [ 10 , 21 , 22 ].…”

“…Many differential diagnoses must be considered, including Behcet’s disease, cellulitis, pyogenic infections, hidradenitis suppurativa, sarcoidosis, tuberculosis, foreign body reactions, contact dermatitis, acquired lymphangiectasia, and sexual abuse before a diagnosis of VCD can be made [ 10 ]. Results of pathological evaluation of gastrointestinal biopsy specimens are vital in making a diagnosis of VCD.…”

Keep CALME (childhood asymmetry labium majus enlargement) and follow up

“…Metastatic Crohn’s disease is a rare extra‐intestinal presentation. Although more common in young adults, the genital involvement in the form of bilateral or asymmetric vulvar swelling has been reported as the first manifestation of Crohn’s disease in children, usually predating the gastrointestinal symptoms [11]. To date, less than 30 cases have been reported in the pediatric population.…”