Demographic and clinical properties of juvenile-onset Behçet's disease: A controlled multicenter study

Accepted 16 October 2007. published online 29 November 2007.

Background

Behçet's disease (BD) is a multisystemic inflammatory disorder of unknown origin. The disease usually occurs between the second and the fourth decades, whereas it is uncommon in children.

Objective

In this multicenter study, we aimed to describe the demographic and clinical features along with severity in juvenile- versus adult-onset BD.

Methods

Patients with initial symptoms at age 16 years or younger were considered as having juvenile-onset BD. In all, 83 patients with juvenile-onset BD (38 male and 45 female; mean age 19.6 ± 7.6 years) and 536 with adult-onset (>16 years) BD (293 male and 243 female; mean age 39.2 ± 10.1 years) who fulfilled the classification criteria of the International Study Group for BD were involved in the study.

Results

Familial cases were more frequent in juvenile-onset compared with adult-onset BD (19% vs 10.3%; P = .017). The mean age of disease onset was 12.29 ± 3.54 years in juvenile-onset BD and 31.66 ± 8.71 years in adult-onset BD. Mucocutaneous lesions and articular symptoms were the most commonly observed manifestations in both groups. The frequency of disease manifestations was not different between juvenile- and adult-onset BD, except neurologic and gastrointestinal involvement, which were higher in juvenile-onset BD than adult-onset BD (P = .027 and P = .024, respectively). Oral ulcer was the most common onset manifestation of both juvenile-onset (86.74%) and adult-onset (89.55%) BD. The frequencies of onset manifestations of BD were similar, except genital ulcer, which was higher in adult-onset BD (P = .025).

Limitations

Our study consisted of patients with BD mainly applying to dermatology and venerology departments. Therefore, it can be speculated that this study includes rather a milder spectrum of the disease.

Conclusions

Although the clinical spectrum of juvenile-onset BD seems to be similar to adult-onset BD, the frequency of severe organ involvement was higher. Because of the higher prevalence of familial cases in juvenile-onset BD, it can be speculated that genetic factors may favor early expression of the disease with severe organ involvement.

Abbreviations used: BD, Behçet's disease, PPL, papulopustular lesions, T1, Time interval between initial symptom and fulfillment of diagnostic criteria, T2, Time interval between fulfillment of criteria and diagnosis

a Department of Dermatology and Venereology at Inonu University School of Medicine, Malatya, Turkey

b Department of Dermatology and Venereology, Erciyes University, School of Medicine, Kayseri, Turkey

c Department of Dermatology and Venereology, Uludag University, School of Medicine, Bursa, Turkey

d Department of Dermatology and Venereology, Akdeniz University, School of Medicine, Antalya, Turkey

e Department of Dermatology and Venereology, Gazi University, School of Medicine, Ankara, Turkey

f Department of Dermatology and Venereology, Cukurova University, School of Medicine, Adana, Turkey

g Department of Dermatology and Venereology, Mersin University, School of Medicine, Mersin, Turkey

h Department of Dermatology and Venereology, Firat University, School of Medicine, Elazig, Turkey

j Department of Dermatology and Venereology, Baskent University Faculty of Medicine, Alanya Hospital, Alanya, Turkey

i Department of Public Health, Akdeniz University School of Medicine, Antalya, Turkey

Reprint requests: Yelda Karincaoglu, MD, İnönü Üniversitesi, Tıp Fakültesi, Dermatoloji AD, 44315 Malatya, Turkey.

Funding sources: None.

Conflicts of interest: None declared.

Presented as free communication at the XII International Conference on Behçet's Disease, in Lisbon, Portugal, on September 20-23, 2006.

PII: S0190-9622(07)02022-1

doi:10.1016/j.jaad.2007.10.452

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