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RESEARCH PRODUCT

Vulvar involvement in pediatric Crohn’s disease: a systematic review

Claudio RomanoGloria CalagnaAntonio PerinoRoberta GraneseGabriele TonniGiuliana MorabitoCarmine Carriero

subject

endocrine systemmedicine.medical_specialtyPediatric Crohn's diseaseVulvar edemaAdministration TopicalAdministration OralDiseasePerineumGastroenterologyVulvaPediatric Crohn’s disease030207 dermatology & venereal diseases03 medical and health sciencesPediatric Crohn’s disease0302 clinical medicineCrohn DiseaseObstetrics and gynaecologyMetronidazoleInternal medicineEdemaHumansMedicineVulvar diseaseChildbusiness.industryObstetrics and GynecologyGeneral MedicineDermatologyExtra-intestinal Crohn’s disease030220 oncology & carcinogenesisVulvar edemaFemaleVulvar DiseasesExtra-intestinal Crohn’s diseasebusinessExtra-intestinal Crohn’s disease; Pediatric Crohn’s disease; Vulvar disease; Vulvar edema; Obstetrics and Gynecology

description

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.

10.1007/s00404-017-4539-xhttp://hdl.handle.net/10447/280428