First Description And Other Observations

The first description of the disease is neither by Adamantiades nor by Behget. Hippokrates of Kos (460-377 B.C.) described an illness whose manifestations resembled very well the cardinal signs of Adamantiades-Behget's disease. Already in the 5th century before Christ in his 3rd "Epidemion" book, case 7 (Fig. 4) he stated: "But there were also other fevers, as it will be described. Many had their mouths affected with aphthous ulcerations. There were also many defluxions about the genital parts, and ulcerations, boils (phymata), externally and internally about the groins. Watery ophthalmies of a chronic character, with pains; fungous excrescences of the eyelids, externally and internally, called fici, which destroyed the sight of many persons. There were fungous growths, in many other instances, on ulcers, especially on those seated on the genital organs. (Many carbuncles grew in the summer as well as other lesions, which were septic, large ecthymata and many large herpetic lesions)"20. It was the ophthalmologist A. Feigenbaum who paid attention to the context of the text quoted above in correspondence with Adamantiades-Behget's disease (ABD)21.

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Figure 4. Original description of the disease by Hippokrates of Kos (text written in Greek, Epidimion book III, case 7)

Already since the 18th century there are reports in international literature describing patients with a symptom complex that resembles ABD. In the historic review presented by Zouboulis and Keitel22 only those case reports or individual cases from larger publications were included which described an illness that can be considered as ABD due to the close similarity of the clinical picture or the obligatory relapsing character of the signs. All 17 patients who have been reported in 10 publications by ophthalmologists presented ocular lesions. Seven of them (41%) presented oral aphthous and genital ulcers while in 12 publications by dermatologists 24 out of 25 patients presented oral aphthous and genital ulcers (96%) but only 3 patients had ocular lesions (12%; p<0.001, chi square test with Yates correction). It is interesting that distinct phenotypes had been reported on male and female patients at that time. While on all male patients ocular lesions (15/15, 100%) were reported, only 5 of the 27 female patients had developed ocular involvement (18.5%, p<0.001, chi square test with Yates correction). On the other hand, in female patients oral aphthous and genital ulcers (25/27, 93%) were more common than in male ones (6/15, 40%, p<0.001). Cutaneous lesions (male 60%, female 63%), mostly erythema nodosum, and arthritis (male 47%, female 15%) were almost equally found in both groups. Indeed, among the 17 patients who had been reported by ophthalmologists were 14 males and 3 females whereas out of 25 patients being presented by dermatologists one was male and 24 female. This finding may explain why ABD was recognized so late as a separate disorder. Ophthalmologists of that time who had mostly followed-up male patients with ocular lesions initially described the disease as "iritis with hypopyon" whereas dermatologists who had followed-up female patients with bipolar mucosal aphthous ulcers used the term "aphthosis". In addition to the term "triple symptom complex" used by Behget18, Dascalopoulos proposed the term "Uveitis recidivans aphthosa"23. Between 1940 and 1950, several cases from different countries were described for the first time22. The Parisian dermatologist A. Touraine described 274 patients with not-infectious oral aphthosis - among them also patients with recurrent iritis with hypopyon -24 in 1941. For the latter cases, Touraine used the term "aphtose généralisée" or "grande aphtose".

Behçet was aware of the publication of Adamantiades and included it in his references. In 1941, Jensen from Denmark, being unaware of Adamantiades' merit, introduced the term "Behçet's syndrome" to describe a patient with the triple symptom complex and ulcerous haemorrhagic colitis and established the pathergy test as a diagnostic criterium25,26. In 1944, Berlin and Ephraim simultaneously described the disorder in necropsy material of a patient from Tel Aviv27 and in a patient from Haifa28, both referring to the important work of Jensen and mentioning the name of Behçet in the titles of their publications. The term "Behçet's disease" was first used in 1946 by Feigenbaum und Kornbluth who considered it a manifestation of a chronic septic condition connected with a constitutional disorder in their description of 4 additional patients from Jerusalem29. Ollendorff Curth, former vice chair of the Department of Dermatology, Virchow Hospital in Berlin (Prof. Buschke), and immigrant to the United States knew the work of Behçet since she had met Behçet in person in Berlin. Her two publications which reported on two American patients30,31 finally made the term "Behçet's syndrome" popular. By reading the first publication of Ollendorff Curth30 it becomes obvious that she had gone through the publications of Jensen, Berlin and Ephraim (which were written in English) but did not have detailed knowledge of Adamantiades' work (written in French) although she included him in her references. Later on, many authors who got access to the work of both authors added the name of Adamantiades to the one of Behçet22.

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