Behçet’s syndrome in pediatric age

Authors

  • Ana Raquel Mendes Department of Pediatrics, Centro Materno-Infantil do Norte, Centro Hospitalar Universitário do Porto
  • Sandrina Braga Department of Vascular Surgery, Hospital da Senhora da Oliveira
  • Catarina Vilarinho Department of Dermatology, Hospital da Senhora da Oliveira
  • Maria Antónia Costa Department of Ophtalmology, Hospital da Senhora da Oliveira
  • Cristina Ferreira Department of Pediatric, Hospital da Senhora da Oliveira
  • Teresa São Simão Department of Pediatric, Hospital da Senhora da Oliveira

DOI:

https://doi.org/10.25753/BirthGrowthMJ.v29.i2.15028

Keywords:

Behçet's syndrome, classification criteria, pediatric age

Abstract

Introduction: Behçet's syndrome is a systemic vasculitis characterized by recurrent oral and/or genital ulcers, and several systemic manifestations. The authors describe the case of a pediatric-onset Behçet's syndrome.
Case report: An 11-year-old boy was referred to the Pediatric consultation after two episodes of great saphenous vein thrombophlebitis. He had experienced daily oral aphthae for the past three years, and various episodes of folliculitis with pustule formation. Laboratory study was normal. The boy showed no signs of uveitis. The diagnosis of Behçet's syndrome diagnosis was established according to the international criteria, with positive HLA- B51 testing. Colchicine was initiated, with favourable response.
Conclusions: Due to clinical feature overlap with other conditions, Behçet's syndrome diagnosis remains challenging. Consensus pediatric classification criteria developed in 2016 enabled greater sensitivity and earlier diagnosis.

Downloads

Download data is not yet available.

References

Koné-Paut I. Behçet´s disease in children, an overview. Pediatric Rheumatology. 2016; 14:10.

Kari JA, Shah V, Dillon MJ. Behçet´s disease in UK children: clinical features and treatment including thalomide. Rheumatology 2001; 40:933-8.

Mahr A, Belarbi L, Wechsler B, Jeanneret D, Dhote R, Fain O, et al. Population-based prevalence study of Behçet´s disease: differences by ethnic origin and low variation by age at immigration. Arthritis Rheum 2008; 58:3951-9.

Calamia KT, Wilson FC, Icen M, Crowson CS, Gabriel SE, Kremers HM. Epidemiology and Clinical Characteristics of Behçet´s disease in the US: a population-based study. Arthritis Rheum 2009; 61:600-4.

Karincaoglu Y, Borlu M, Toker SC, Akman A, Onder M, Gunasti S, et al. Demographic and clinical properties of juvenile-onset Behçet´s disease: A controlled multi-center study. J Am Acad Dermatol 2008; 58:579-84.

Direskeneli H. Behçet´s disease: infectious aetiology, new autoantigens, and HLA-B51. Ann Rheum Dis 2001; 60:996-1002.

International Study group for Behçet´s disease. Criteria for diagnosis of Behçet´s disease. Lancet. 1990; 335:1078-80.

International Team for the Revision of the International Criteria for Behçet´s disease (ITR-ICBD). The International Criteria for Behçet´s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014; 28:338-47.

Koné-Paut I, Shahram F, Darce-Bello M, Cantarini L, Cimaz R, Gattorno M, et al. Consensus classification criteria for paediatric Behçet´s disease from a prospective observational cohort: PEDBD. Ann Rheum Dis. 2016; 75:958-64.

Kokturk A. Clinical and Pathological Manifestations with Differential Diagnosis in Behçet´s disease. Patholog Res Int. 2012; 2012:690390.

Koné-Paut I, Yurdakul S, Bahabri SA, Shafae N, Ozen S, Ozdogan H, et al. Clinical features of Behçet´s disease in children: an international collaborative study of 86 cases. J Pediatr. 1998; 132:721-5.

Altiparmak MR, Tanverdi M, Pamuk ÚN, Tunç R, Hamuryudan V. Glomerulonephritis in Behçet´s disease: Report of seven cases and review of literature. Clin Rheumatol. 2002; 21:14-8.

Koné-Paut I, Geisler I, Wechsler B, Ozen S, Ozdogan H, Rozenbaum M, et al. Familial aggregation in Behçet´s disease: high frequency in siblings and parents of pediatric probands. J Pediatr. 1999; 135:89-93.

Saadoun D, Wechsler B, Desseaux K, Le Thi Huong D, Amoura Z, Resche-Rigon M, et al. Mortality in Behçet´s disease. Arthritis Rheum. 2010; 62:2806-12.

Hatemi G, Christensen R, Bang Dongsik, Bodaghi B, Celik AF, Fortune F, et al. 2018 Update of the EULAR recommendations for the management of Behçet´s syndrome. Ann Rheum Dis 2018; 77:808-18.

Downloads

Published

2020-06-26

How to Cite

1.
Mendes AR, Braga S, Vilarinho C, Costa MA, Ferreira C, Simão TS. Behçet’s syndrome in pediatric age. REVNEC [Internet]. 2020Jun.26 [cited 2024Aug.19];29(2):121-5. Available from: https://revistas.rcaap.pt/nascercrescer/article/view/15028

Issue

Vol. 29 No. 2 (2020)

Section

Case Reports

License

Copyright and access

This journal offers immediate free access to its content, following the principle that providing free scientific knowledge to the public provides greater global democratization of knowledge.
The works are licensed under a Creative Commons Attribution Non-commercial 4.0 International license.
Nascer e Crescer – Birth and Growth Medical Journal do not charge any submission or processing fee to the articles submitted.

Most read articles by the same author(s)