Purpose: The aim of the study is to report a systematic review (from 2000 to 2017) of all pediatric cases of vulvar Crohn's disease (VCD) and to highlight the key-points for a correct diagnosis and management of this rare condition.
Methods: An electronic search using the Pubmed/Medline, Scopus, EMBASE, Cochrane database and Google Scholar database was performed according to PRISMA guidelines.
Results: Twenty pediatric studies and 22 cases of VCD were included for analysis. All the articles reported a single case, except two articles where two cases, respectively, are described. Clinical vulvar examination showed the following main manifestations: vulvar erythema (9/22 cases, 40.9%), vulvar swelling (8/22 cases, 36.4%), vulvar edema (8/22 cases, 36.4%), vulvar ulcers (4/22 cases, 18.2%). Perianal and/or anal involvement (fissures, vegetations, skin tags, erythema, papules, nodules) were recorded in ten cases (45.4%). Steroids per os and/or topical administration were the most prescribed treatment, achieving clinical remission in 11 cases (50%), used alone or in combination with metronidazole or 5-aminosalicylic acid, azathioprine or sulphasalazine/mesalazine.
Conclusions: This review shows that pediatric VCD is an uncommon disease, difficult to be diagnosed as either symptoms or clinical lesions are not specific. A multidisciplinary approach is advised to reach a correct diagnosis and plan clinical treatment.
Keywords: Extra-intestinal Crohn’s disease; Pediatric Crohn’s disease; Vulvar disease; Vulvar edema.