Twenty-eight cases of juvenile-onset Adamantiades-Behçet disease in Germany.
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.
Treudler
R
R
Department of Dermatology, University Medical Center Benjamin Franklin, The Free University of Berlin, Berlin, Germany.
Orfanos
C E
CE
Zouboulis
C C
CC
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D001528:Behcet Syndrome / Q000453:epidemiology / Q000378:metabolism / Q000473:pathology*; D002648:Child; D005260:Female; D005858:Germany / Q000453:epidemiology; D015235:HLA-B Antigens / Q000032:analysis; D059949:HLA-B51 Antigen; D006801:Humans; D008297:Male; D015995:Prevalence; D012867:Skin / Q000737:chemistry / Q000473:pathology*; D013281:Stomatitis, Aphthous / Q000473:pathology
D015235:HLA-B Antigens; D059949:HLA-B51 Antigen