[Neonatal Behçet Disease].

KLINISCHE PADIATRIE(2018)

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摘要
Background Behcet's disease is a multi-system disease with inflammatory infestations, in particular of the small and middle arteries and veins. Histologically, the disease presents with the image of leukocytoclastic vasculitis. The etiology of Behcet disease is unknown, but genetic factors are discussed of playing a role in its pathogenesis. There is no known autoantibody, which could serve as a diagnostic tool. The disease usually affects patients in the 3. life decade and is rare in pediatrics. Avery rare clinical form of Behcet's disease occurs during neonatal period. In this form life-threatening courses of the disease have been described. Patient/Method In this report a family with a diagnosed disorder of the mother, where the second child presented with ulcerations in the mucous membrane area during the first week of life, will be discussed. In this case, the question of a transfer of autoantibodies from the mother, which triggers the disease in the child, must be raised. The clinical progress will be compared to known cases in the literature in order to derive recommendations for a therapeutic procedure. Results In our patient, ANAs were transiently detected, which correlated to the mother's. Fortunately, the course of the disease was mild, so that no further treatment was necessary. But, potential life-threatening events should be always held in consideration. Here, steroids seem to be the treatment of choice. Conclusion In the wake of the increasing immigration of pregnant Morbus Behcet patients from Mediterranean and from countries with higher prevalence of Morbus Behcet-positive mothers should be considered.
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关键词
Neonatal Behcet disease,neonatology,leukoclastic vasculitis,antibody,ulceration
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