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A fter the publication of an article on “Behçet’s disease and thrombophilia” by Leiba et al,1 Ehrlich2 and Leiba et al3 discussed descriptions of the disease before Behçet. They agreed that Behçet was the first modern author to group the various ophthalmic, dermatological, and orogenital lesions together as a syndrome.
However, during the annual meeting of the Medical Society of Athens on 15 November 1930, Benediktos Adamantiades (1875–1962),4–6 Greek ophthalmologist from Prussa, Asia minor (nowadays Bursa, Turkey), presented a lecture entitled “A case of relapsing iritis with hypopyon”, describing a 20 year old male patient with the three cardinal signs of the disease. The disease had begun at the age of 18 with oedema and ulcerations at the left leg diagnosed as thrombophlebitis. During the following two years (1928–30) the patient developed recurrent iritis with hypopyon in both eyes, which led to blindness and atrophy of the optic nerve, scrotal ulcers healing with scars, oral aphthous ulcers, and a sterile arthritis of both knees. The last three signs were recurrent. Bacterial cultures of knee and anterior eye chamber punctures were sterile and the inoculation experiments in animals were negative, while staphylococci had grown in cultures from scrotal ulcers and a tonsilar abscess.
In the same year the lecture was published in the Proceedings of the Medical Society of Athens7 and in the following year in the French journal Annales d’Oculistique.8 Adamantiades connected the genital ulcers, the arthritis, and the ocular disease as signs of a single disease. He referred to publications by Reis (1906) and Gilbert (1920), who described similar cases, and backed the hypothesis of a bacterial, focal illness induced by staphylococci, which had been initiated by Gilbert (1925).9 He also responded to the comment of the physician V Vasilopoulos that the case can be classified as the already described, so-called “metastatic ophthalmias”, which indicate a generalised infection, stating “I would like to comment to my colleague that the disease which reports Gilbert as aetiological (notice: induced by staphylococci) does not correlate with all types of iritis, but to this iritis with recurrent hypopyon, which now constitutes itself a clinical entity” (fig 1).
In 1946 Adamantiades reported on two further patients and defined thrombophlebitis as the fourth cardinal sign of the disease.10 Later, he presented the first classification of the disease by describing the ocular, mucocutaneous, and systemic forms in a review.11 He pointed out that the disease can occur for years as a monosymptomatic or oligosymptomatic disorder and that eye involvement and severe prognosis are more common in men than in women. In this work he also proposed the first diagnostic criteria.
The term “Adamantiades-Behçet’s disease” honours both men who first described the several manifestations which constitute an autonomous disease and is, therefore, proposed for naming this disorder.