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Published in: Clinical Rheumatology 12/2011

01-12-2011 | Case Based Review

Neonatal Behçet’s disease without maternal history

Authors: Yung-Sen Chang, Yao-Hsu Yang, Bor-Luen Chiang

Published in: Clinical Rheumatology | Issue 12/2011

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Abstract

A neonate presented with frequent vomiting since 10 days old, followed by severe diarrhea. Multiple oral ulcers and recurrent genital ulcers subsequently appeared. Colonoscopy showed multiple shallow round ulcerations in the colon. The symptoms responded well to a short course of oral prednisolone. There was a brief recurrence of vomiting, diarrhea, and oral ulcers at 2 months old. The mother has never had any symptoms of Behçet's disease to date. This is the first reported case in literature of neonatal Behçet's disease without a maternal history of Behçet's disease, which raises doubts about the assumed role of maternal antibodies in the pathogenesis of neonatal Behçet's disease. A literature review of neonatal Behçet's disease shows that oral ulcers, skin lesions, fever, and leukocytosis are common features. However, only half of the patients fulfill the classical diagnostic criteria based on adult studies. A treatment consensus for neonatal cases is also lacking.
Literature
2.
go back to reference Fam AG, Siminovitch KA, Carette S, From L (1981) Neonatal Behçet's syndrome in an infant of a mother with the disease. Ann Rheum Dis 40(5):509–512PubMedCrossRef Fam AG, Siminovitch KA, Carette S, From L (1981) Neonatal Behçet's syndrome in an infant of a mother with the disease. Ann Rheum Dis 40(5):509–512PubMedCrossRef
3.
4.
go back to reference Stark AC, Bhakta B, Chamberlain MA, Dear P, Taylor PV (1997) Life-threatening transient neonatal Behçet's disease. Br J Rheumatol 36(6):700–702PubMedCrossRef Stark AC, Bhakta B, Chamberlain MA, Dear P, Taylor PV (1997) Life-threatening transient neonatal Behçet's disease. Br J Rheumatol 36(6):700–702PubMedCrossRef
5.
go back to reference Thivolet J, Cambazard F, Genvo MF (1982) Maternally-transmitted severe neonatal aphthosis. Ann Dermatol Venereol 109(9):815–816PubMed Thivolet J, Cambazard F, Genvo MF (1982) Maternally-transmitted severe neonatal aphthosis. Ann Dermatol Venereol 109(9):815–816PubMed
8.
go back to reference Aksu K, Kabasakal Y, Sayiner A, Keser G, Oksel F, Bilgic A, Gumusdis G, Doganavsargil E (1999) Prevalences of hepatitis A, B, C and E viruses in Behçet's disease. Rheumatology (Oxford) 38(12):1279–1281CrossRef Aksu K, Kabasakal Y, Sayiner A, Keser G, Oksel F, Bilgic A, Gumusdis G, Doganavsargil E (1999) Prevalences of hepatitis A, B, C and E viruses in Behçet's disease. Rheumatology (Oxford) 38(12):1279–1281CrossRef
11.
go back to reference Ersoy O, Ersoy R, Yayar O, Demirci H, Tatlican S (2007) H pylori infection in patients with Behçet's disease. World J Gastroenterol 13(21):2983–2985PubMed Ersoy O, Ersoy R, Yayar O, Demirci H, Tatlican S (2007) H pylori infection in patients with Behçet's disease. World J Gastroenterol 13(21):2983–2985PubMed
12.
go back to reference Isogai E, Hirata M, Isogai H, Matuo K, Kimura K, Yokota K, Oguma K, Tojo M, Kaneko F, Kotake S, Ohno S (2003) Antimicrobial activity of synthetic human CAP18 peptides to Streptococcus sanguis isolated from patients with Behçet's disease. Adv Exp Med Biol 528:195–200. doi:10.1007/0-306-48382-3_38 PubMedCrossRef Isogai E, Hirata M, Isogai H, Matuo K, Kimura K, Yokota K, Oguma K, Tojo M, Kaneko F, Kotake S, Ohno S (2003) Antimicrobial activity of synthetic human CAP18 peptides to Streptococcus sanguis isolated from patients with Behçet's disease. Adv Exp Med Biol 528:195–200. doi:10.​1007/​0-306-48382-3_​38 PubMedCrossRef
13.
go back to reference Karacayli U, Mumcu G, Simsek I, Pay S, Kose O, Erdem H, Direskeneli H, Gunaydin Y, Dinc A (2009) The close association between dental and periodontal treatments and oral ulcer course in Behçet's disease: a prospective clinical study. J Oral Pathol Med 38(5):410–415. doi:10.1111/j.1600-0714.2009.00765.x PubMedCrossRef Karacayli U, Mumcu G, Simsek I, Pay S, Kose O, Erdem H, Direskeneli H, Gunaydin Y, Dinc A (2009) The close association between dental and periodontal treatments and oral ulcer course in Behçet's disease: a prospective clinical study. J Oral Pathol Med 38(5):410–415. doi:10.​1111/​j.​1600-0714.​2009.​00765.​x PubMedCrossRef
14.
go back to reference Zervas J, Vayopoulos G, Sakellaropoulos N, Kaklamanis P, Fessas P (1988) HLA antigens and Adamantiades-Behçet's disease (A-BD) in Greeks. Clin Exp Rheumatol 6(3):277–280PubMed Zervas J, Vayopoulos G, Sakellaropoulos N, Kaklamanis P, Fessas P (1988) HLA antigens and Adamantiades-Behçet's disease (A-BD) in Greeks. Clin Exp Rheumatol 6(3):277–280PubMed
15.
17.
go back to reference Yasuoka H, Yamaguchi Y, Mizuki N, Nishida T, Kawakami Y, Kuwana M (2008) Preferential activation of circulating CD8+ and gammadelta T cells in patients with active Behçet's disease and HLA-B51. Clin Exp Rheumatol 26(4 Suppl 50):S59–S63PubMed Yasuoka H, Yamaguchi Y, Mizuki N, Nishida T, Kawakami Y, Kuwana M (2008) Preferential activation of circulating CD8+ and gammadelta T cells in patients with active Behçet's disease and HLA-B51. Clin Exp Rheumatol 26(4 Suppl 50):S59–S63PubMed
18.
go back to reference Zouboulis CC, Katsantonis J, Ketteler R, Treudler R, Kaklamani E, Hornemann S, Kaklamanis P, Orfanos CE (2000) Adamantiades-Behçet's disease: interleukin-8 is increased in serum of patients with active oral and neurological manifestations and is secreted by small vessel endothelial cells. Arch Dermatol Res 292(6):279–284PubMedCrossRef Zouboulis CC, Katsantonis J, Ketteler R, Treudler R, Kaklamani E, Hornemann S, Kaklamanis P, Orfanos CE (2000) Adamantiades-Behçet's disease: interleukin-8 is increased in serum of patients with active oral and neurological manifestations and is secreted by small vessel endothelial cells. Arch Dermatol Res 292(6):279–284PubMedCrossRef
20.
go back to reference Leiba M, Sidi Y, Gur H, Leiba A, Ehrenfeld M (2001) Behçet's disease and thrombophilia. Ann Rheum Dis 60(12):1081–1085PubMedCrossRef Leiba M, Sidi Y, Gur H, Leiba A, Ehrenfeld M (2001) Behçet's disease and thrombophilia. Ann Rheum Dis 60(12):1081–1085PubMedCrossRef
21.
go back to reference Lee KH, Chung HS, Kim HS, Oh SH, Ha MK, Baik JH, Lee S, Bang D (2003) Human alpha-enolase from endothelial cells as a target antigen of anti-endothelial cell antibody in Behçet's disease. Arthritis Rheum 48(7):2025–2035. doi:10.1002/art.11074 PubMedCrossRef Lee KH, Chung HS, Kim HS, Oh SH, Ha MK, Baik JH, Lee S, Bang D (2003) Human alpha-enolase from endothelial cells as a target antigen of anti-endothelial cell antibody in Behçet's disease. Arthritis Rheum 48(7):2025–2035. doi:10.​1002/​art.​11074 PubMedCrossRef
23.
25.
go back to reference International Study Group for Behcet's Disease (1990) Criteria for diagnosis of Behçet's disease. Lancet 335(8697):1078–1080 International Study Group for Behcet's Disease (1990) Criteria for diagnosis of Behçet's disease. Lancet 335(8697):1078–1080
26.
go back to reference Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, Gul A, Houman MH, Kotter I, Olivieri I, Salvarani C, Sfikakis PP, Siva A, Stanford MR, Stubiger N, Yurdakul S, Yazici H (2008) EULAR recommendations for the management of Behçet disease. Ann Rheum Dis 67(12):1656–1662. doi:10.1136/ard.2007.080432 PubMedCrossRef Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, Gul A, Houman MH, Kotter I, Olivieri I, Salvarani C, Sfikakis PP, Siva A, Stanford MR, Stubiger N, Yurdakul S, Yazici H (2008) EULAR recommendations for the management of Behçet disease. Ann Rheum Dis 67(12):1656–1662. doi:10.​1136/​ard.​2007.​080432 PubMedCrossRef
27.
go back to reference Shimizu T, Ehrlich GE, Inaba G, Hayashi K (1979) Behçet disease (Behçet syndrome). Semin Arthritis Rheum 8(4):223–260PubMedCrossRef Shimizu T, Ehrlich GE, Inaba G, Hayashi K (1979) Behçet disease (Behçet syndrome). Semin Arthritis Rheum 8(4):223–260PubMedCrossRef
28.
go back to reference Chen YC, Chang HW (2001) Clinical characteristics of Behçet's disease in southern Taiwan. J Microbiol Immunol Infect 34(3):207–210PubMed Chen YC, Chang HW (2001) Clinical characteristics of Behçet's disease in southern Taiwan. J Microbiol Immunol Infect 34(3):207–210PubMed
29.
go back to reference Fujikawa S, Suemitsu T (1997) Behçet disease in children: a nationwide retrospective survey in Japan. Acta Paediatr Jpn 39(2):285–289PubMed Fujikawa S, Suemitsu T (1997) Behçet disease in children: a nationwide retrospective survey in Japan. Acta Paediatr Jpn 39(2):285–289PubMed
Metadata
Title
Neonatal Behçet’s disease without maternal history
Authors
Yung-Sen Chang
Yao-Hsu Yang
Bor-Luen Chiang
Publication date
01-12-2011
Publisher
Springer-Verlag
Published in
Clinical Rheumatology / Issue 12/2011
Print ISSN: 0770-3198
Electronic ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-011-1830-2

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