Asymptomatic Granulomatous Vulvitis and Granulomatous Cheilitis in Childhood: The Need for Crohn Disease Workup

UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ, USA.
Journal of pediatric gastroenterology and nutrition (Impact Factor: 2.63). 07/2011; 53(1):100-1. DOI: 10.1097/MPG.0b013e31820bcff9
Source: PubMed


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    ABSTRACT: Granulomatous cheilitis and granulomatous vulvitis are rare disorders characterized by painless swelling of lips and vulva, respectively. Histopathology of both conditions show non-caseating epithelioid cell granulomas in the dermis. Both disorders have been associated with Crohn's disease rarely. Occurrence of the two conditions in the same patient is extremely infrequent. We hereby report, the association of granulomatous cheilitis with granulomatous vulvitis in a 30-year-old female.
    Indian journal of dermatology, venereology and leprology 11/2013; 79(6):799-801. DOI:10.4103/0378-6323.120733 · 1.39 Impact Factor
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    ABSTRACT: Aim: To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn's disease. Methods: We conducted a systematic review according to the PRISMA guidelines. We searched Medline, LILACS, Virtual Health Library, and Web of Knowledge in September 2013 for cases of OFG in the pediatric age range (< 18 years), with no language limitations. All relevant articles were accessed in full text. The manual search included references of retrieved articles. We extracted data on patients' characteristics, disease characteristics, association with other diseases, and treatment. We analyzed the data and reported the results in tables and text. Results: We retrieved 173 reports of OFG in children. Mean age at onset was 11.1 ± 3.8 years (range: 2.0-18 years). Prevalence in males was significant higher than in females (P < 0.001), with a male:female ratio of 2:1. Gastrointestinal signs or symptoms were present in 26.0% of children at the time of OFG diagnosis. Overall, 70/173 (40.4%) children received a concomitant diagnosis of Crohn's disease. In about half (51.4%) of the cases the onset of OFG anticipated the diagnosis of Crohn's disease, with a mean time between the two diagnoses of 13.1 ± 11.6 mo (range: 3-36 mo). Overall, 21/173 (12.1%) of the children with OFG had perianal disease, while 11/173 (6.4%) had a family history of Crohn's disease. Both perianal disease and a family history of Crohn's disease were significantly associated with a higher risk of Crohn's disease diagnosis in children with OFG [relative risk (RR) = 3.10, 95% confidence interval (CI): 2.46-3.90; RR = 2.74, 95%CI: 2.24-3.36, P < 0.0001 for both). Treatment of OFG included steroids (70.8% of children) and other immunosuppressive drugs (42.7%), such as azathioprine, thalidomide and infliximab. Conclusion: High prevalence of Crohn's disease in children with OFG suggests that OFG may be a subtype of Crohn's disease.
    World Journal of Gastroenterology 06/2014; 20(23):7497-504. DOI:10.3748/wjg.v20.i23.7497 · 2.37 Impact Factor