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78 Transient neonatal Behçet’s disease: a case report

BACKGROUND: Behçet’s disease (BD) is a systemic vasculitis of unknown aetiology. The disease usually affects patients in the 3rd life decade and is rare in pediatrics. A very rare clinical form of Behçet's disease occurs during neonatal period. OBSERVATION: We report a case of a term neonate pr...

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Autores principales: Hatem, Rouag, Marwa, El-Ouaer, Salma, Dhouib, Zaineb, Ben Mohamed, Ghofrane, Bedoui, Siwar, Abdelmoula, Hayet, Ben Hmida, Kamel, Monastiri
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9538992/
http://dx.doi.org/10.1093/rheumatology/keac496.074
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author Hatem, Rouag
Marwa, El-Ouaer
Salma, Dhouib
Zaineb, Ben Mohamed
Ghofrane, Bedoui
Siwar, Abdelmoula
Hayet, Ben Hmida
Kamel, Monastiri
author_facet Hatem, Rouag
Marwa, El-Ouaer
Salma, Dhouib
Zaineb, Ben Mohamed
Ghofrane, Bedoui
Siwar, Abdelmoula
Hayet, Ben Hmida
Kamel, Monastiri
author_sort Hatem, Rouag
collection PubMed
description BACKGROUND: Behçet’s disease (BD) is a systemic vasculitis of unknown aetiology. The disease usually affects patients in the 3rd life decade and is rare in pediatrics. A very rare clinical form of Behçet's disease occurs during neonatal period. OBSERVATION: We report a case of a term neonate presenting with oral and genital ulcerations appeared at the age of 7 days. The mother had a history of Behçet’s disease and antiphospholipid antibody syndrome (APLS) diagnosed at the age of 20 years, characterized by severe recurrent orogenital ulceration and complicated by thrombophlebitis and mycocarditis. She was treated with colchicine, corticoids during the attacks and an antiplatelet treatment. Also, the siblings of the neonate (2 sisters and one brother) had the same symptoms in the neonatal period and developed disfiguring scars since they were treated as herpes by antiviral therapy. Family history, clinical course and negative laboratory results, suggest the diagnosis of transient neonatal BD. The treatment with systemic and local corticoids was effective. The neonate didn’t develop scars, neurological or vascular symptoms. The antibodies anti-nuclear and HLAB51 were negative. CONCLUSION: Transient neonatal Behçet’s disease (BD) is a rare secondary neonatal autoimmune condition. The transmission from mother to fetus is still unclear. It is thought to be transmitted by an immune mechanism. However, the causative antibodies for neonatal BD have not been identified. Corticosteroid is recommended after diagnosis in order to avoid scars
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spelling pubmed-95389922022-10-07 78 Transient neonatal Behçet’s disease: a case report Hatem, Rouag Marwa, El-Ouaer Salma, Dhouib Zaineb, Ben Mohamed Ghofrane, Bedoui Siwar, Abdelmoula Hayet, Ben Hmida Kamel, Monastiri Rheumatology (Oxford) E POSTERS BACKGROUND: Behçet’s disease (BD) is a systemic vasculitis of unknown aetiology. The disease usually affects patients in the 3rd life decade and is rare in pediatrics. A very rare clinical form of Behçet's disease occurs during neonatal period. OBSERVATION: We report a case of a term neonate presenting with oral and genital ulcerations appeared at the age of 7 days. The mother had a history of Behçet’s disease and antiphospholipid antibody syndrome (APLS) diagnosed at the age of 20 years, characterized by severe recurrent orogenital ulceration and complicated by thrombophlebitis and mycocarditis. She was treated with colchicine, corticoids during the attacks and an antiplatelet treatment. Also, the siblings of the neonate (2 sisters and one brother) had the same symptoms in the neonatal period and developed disfiguring scars since they were treated as herpes by antiviral therapy. Family history, clinical course and negative laboratory results, suggest the diagnosis of transient neonatal BD. The treatment with systemic and local corticoids was effective. The neonate didn’t develop scars, neurological or vascular symptoms. The antibodies anti-nuclear and HLAB51 were negative. CONCLUSION: Transient neonatal Behçet’s disease (BD) is a rare secondary neonatal autoimmune condition. The transmission from mother to fetus is still unclear. It is thought to be transmitted by an immune mechanism. However, the causative antibodies for neonatal BD have not been identified. Corticosteroid is recommended after diagnosis in order to avoid scars Oxford University Press 2022-10-07 /pmc/articles/PMC9538992/ http://dx.doi.org/10.1093/rheumatology/keac496.074 Text en © The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle E POSTERS
Hatem, Rouag
Marwa, El-Ouaer
Salma, Dhouib
Zaineb, Ben Mohamed
Ghofrane, Bedoui
Siwar, Abdelmoula
Hayet, Ben Hmida
Kamel, Monastiri
78 Transient neonatal Behçet’s disease: a case report
title 78 Transient neonatal Behçet’s disease: a case report
title_full 78 Transient neonatal Behçet’s disease: a case report
title_fullStr 78 Transient neonatal Behçet’s disease: a case report
title_full_unstemmed 78 Transient neonatal Behçet’s disease: a case report
title_short 78 Transient neonatal Behçet’s disease: a case report
title_sort 78 transient neonatal behçet’s disease: a case report
topic E POSTERS
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9538992/
http://dx.doi.org/10.1093/rheumatology/keac496.074
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