[Epidemiology and clinical manifestations of Adamantiades-Behçet disease in Germany -- current pathogenetic concepts and therapeutic possibilities].

© Blackwell Verlag GmbH • www.blackwell.de • 1610-0379/2006/0401-0049 JDDG | 1 ̇2006 (Band 4) Introduction Historical background and nomenclature In his third book of “Epidimion” Hippocrates of Kos (460–377 BC) described a disease with symptoms comparable to the cardinal symptoms of Adamantiades-Behçet disease (ABD): “Many patients had aphthous ulcerations in the mouth. There were numerous purulent genital ulcers, as well as furuncles, which were external and internal, as well as in the groins. There was a watery inflammation of the eye that was chronic and painful as well as growths on the eyelids – external and internal – which impaired eyesight in many patients. (...) There were also larger ecthymata and large herpetic lesions in many patients. “ In 1930 Benediktos Adamantiades (1875–1965), a Greek ophthalmologist, presented a lecture describing findings in an 18-year-old patient, for the first time grouping ocular symptoms, oral aphthae, and genital ulcers together as symptoms of a single clinical entity. He later added vascular involvement as a fourth cardinal symptom and in a review article presented the first classification of the disease by describing its ocular, mucocutaneous, and systemic forms [1]. The chief symptom, which according to Adamantiades was hypopyon, appears at various intervals, causing more or less lasting permanent damage and ultimately resulting in blindness. Hulûsi Behçet (1889–1948), a Turkish dermatologist, first described in 1937 patients with recurrent lesions affecting the mouth, genitals, and eyes, adding a catalogue of symptoms including periodontitis, jaw cysts, acneiform skin changes, and erythema nodosum as well as arthralgia. Behçet’s intense work on the disease and his conviction about its autonomous character finally brought this puzzling clinical picture to the attention of the scientific community. CME