E-Mail karger@karger.com Review Paper Dermatology 2016;232:129–136 DOI: 10.1159/000442667 Juvenile Adamantiades-Behçet Disease Aristeidis G. Vaiopoulos a Meletios A. Kanakis c Violetta Kapsimali d Georgios Vaiopoulos c Phedon G. Kaklamanis e Christos C. Zouboulis b a Institute of Pathology, University of Würzburg, Würzburg, and b Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, Dessau, Germany; c Institute of Experimental Physiology and d Department of Microbiology, Medical School, University of Athens, and e Athens Medical Center, Athens, Greece without a clear outcome to date [1, 3]. It is a worldwide disease with a predilection for people living in the Far East, Middle East and the Mediterranean regions (former so- called Silk Route). ABD affects people of all ages, showing the highest prevalence of onset in the third decade of life. Both genders may be involved, though its clinical spectrum and severity display quite substantial differences between them [4, 5]. Patients with symptoms occurring up to the age of 16 are considered as cases of juvenile-onset ABD (JABD) [6, 7]. JABD is relatively rare compared to its adult counter- part (AABD). Since the publication of the first article on the pediatric disease by Mundy and Miller in 1978 [8], several case reports and case studies have been described [6, 7, 9–14]. However, reviews on this subject are rather scarce [6, 15–18]. Epidemiology The epidemiology of JABD is difficult to estimate also because there is no formal agreement on either the age at the disease onset or the age at which the symptoms meet the older or current diagnostic and classification criteria [19, 20]. Key Words Aphthae · Childhood · Epidemiological study · Genitoanal region · Adamantiades-Behçet disease · Behçet’s disease · Uveitis Abstract Adamantiades-Behçet disease (ABD) is a chronic, multisys- temic, recurrent, inflammatory vascular disorder of unknown etiology. Patients with symptoms initially appearing at the age of 16 or less are considered as cases of juvenile-onset ABD (JABD). JABD is relatively rare compared to ABD of adults, and only case reports and case studies have been published regarding this subtype of the disease. Epidemiol- ogy, clinical features, diagnosis and treatment of JABD are discussed in this review. © 2016 S. Karger AG, Basel Introduction Adamantiades-Behçet disease (ABD) is a chronic, mul- tisystemic, recurrent, inflammatory vascular disorder of unknown etiology [1, 2]. Genetic or environmental factors as well as immunological aberrations have been incrimi- nated by various investigators for its etiopathogenesis, still Received: September 4, 2015 Accepted after revision: November 20, 2015 Published online: January 7, 2016 Prof. Dr. med. Prof. h.c. Dr. h.c. Christos C. Zouboulis Departments of Dermatology, Venereology, Allergology and Immunology Dessau Medical Center Auenweg 38, DE–06847 Dessau (Germany) E-Mail christos.zouboulis  @  klinikum-dessau.de © 2016 S. Karger AG, Basel 1018–8665/16/2322–0129$39.50/0 www.karger.com/drm