Twenty-eight cases of juvenile-onset Adamantiades-Behçet disease in Germany

Dermatology. 1999;199(1):15-9. doi: 10.1159/000018197.

Abstract

Background and objective: Adamantiades-Behçet disease is a rare entity at a juvenile age. We aimed to enlighten epidemiological and clinical characteristics of juvenile-onset disease in Germany.

Methods: Data from the German Registry were used to compare clinical and epidemiological features of patients with juvenile-onset (</=16 years) and adult-onset (>16 years) disease diagnosed according to the criteria of the International Study Group.

Results: Twenty-eight (17%) of 168 patients of the German Registry exhibited the onset of the disease and 8 (5%) of them the complete symptom complex at a juvenile age. Juvenile-onset disease was characterized by an increase in familial cases (25 vs. 8% in patients with adult-onset; p = 0.047). The frequency of diagnostic signs was similar between the two study groups. In juvenile-onset disease, delayed development of the complete symptom complex (median value 35 months vs. 12 months after onset; p = 0.014) and lower prevalence of severe complications (9 vs. 29%; p = 0.042) were detected.

Conclusions: The major clinical features of juvenile-onset and adult-onset disease in Germany are comparable, but in juvenile-onset disease, the course is delayed and patients experience less severe complications. In addition, there is a higher rate of familial occurrence of the disease in patients with juvenile-onset.

Publication types

  • Comparative Study
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Behcet Syndrome / epidemiology
  • Behcet Syndrome / metabolism
  • Behcet Syndrome / pathology*
  • Child
  • Female
  • Germany / epidemiology
  • HLA-B Antigens / analysis
  • HLA-B51 Antigen
  • Humans
  • Male
  • Prevalence
  • Skin / chemistry
  • Skin / pathology*
  • Stomatitis, Aphthous / pathology

Substances

  • HLA-B Antigens
  • HLA-B51 Antigen