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Medicina Oral, Patología Oral y Cirugía Bucal (Internet)

On-line version ISSN 1698-6946

Med. oral patol. oral cir.bucal (Internet) vol.11 n.5  Aug./Sep. 2006



Dear Editor of Medicina Oral, Patología Oral y Cirugía Bucal,

I have read the very recent paper of Eguia A et al. (Med Oral Patol Oral Cir Bucal 2006;11:E6-11) with great interest titled "Adamantiades-Behçet disease: an enigmatic process with oral manifestations". In their paper, the authors evaluated the main etiological, clinical and therapeutic aspects of Behçet disease.

As a "Behçetologist" and an author with 26 published original articles on Behçet disease in internatonal medical journals,1 I was very disappointed to read that the syndrome was individually named as "Adamantiades–Behçet" in the title and throughout the text. The title of the table 1 on page E9, for instance, states "Diagnostic criteria for ABD". There is no such a diagnostic criteria we know in the literature claimed by the authors. On the contrary, there are only two internationally accepted criteria; (1) International Study Group Criteria for the diagnosis of Behçet disease(2) (not Adamantiades-Behçet disease or ABD) and (2) the criteria of "The Behçet’s Disease Research Committee of Japan".(3)

Therefore, I would be very appreciated if the authors of the present paper strongly address the reasons (1) why they are trying to change of an old and established disorder well–known as "Behçet disease" since 1941 not only by every physician interested in Behçet disease care in the world, but also by the medical students and even public, and (2) why should only Dr. Benedictos Adamantiades be honored among all faithful authors who reported individual cases with one or more Behçet symptoms from the beginning of Hippocratic writings more than 2000 years ago until the individual case of Dr. Adamantiades.

Strictly speaking, in 1930, Dr. Adamantiades reported a male patient with recurrent hypopyon iritis and orogenital ulcers.(4) In the etiology, he ascribed the findings to another disease such as syphilis, tuberculosis, and staphylococcal bacteremia, resulting in treatment of his patient by anti–syphilitic medications, though the disease was not healed.(5) Adamantiades cited the publications of Reis, Gilbert, and Weve in his paper, all of whom also accused such infections in the etiology.(6) Dr. Adamantiades then suggested from the ophthalmologist perspective that "recurrent iritis" may be a distinct entity, which, indeed, may occur not only in many systemic infective and noninfective disorders, but also in ocular or non-ocular diseases or even after some uveitogenic medications (see Table 4 please of our major review article in Survey for differential diagnosis of these symptoms).(1) Therefore, as International Behçet Society also indicated, Adamantiades’ patient was not the first with classical triad, he did not recognize the true nature of the disease and did not presented these manifestations as "triple symptom complex" indicating as a new and distinct clinical entity.(5-7)

If Dr. Eguia A et al.’s thesis that is stated in the second paragraph of the introduction section of the paper is to be accepted as a reason for the suggested naming, a more appropriate new full name should be "Hippocrates–…–Janin–Neumann–Christlieb–Reis–Blüthe–Gilbert–Planner–Remenowsky–Weve–Shigeta–Pils–Grütz–Carol –Ruys–Samek–Fisher–Walter–Roman–Kumer–Adamantiades–Behçet disease", though several other authors remained still unnamed here.(8) Indeed, all these above-listed authors, like Adamantiades did in 1930, reported one or many symptoms or signs of Behçet’s findings including recurrent oral aphthae, genital ulcerations, some other mucocutaneous lesions including erythema nodosum, thrombophlebitis as well as arthritis along with recurrent iritis with or without hypopyon uveitis. Among these publications, for instance, "recurrent uveitic lesions" (a major criteria in both "International Study Group Criteria" of Behçet disease(2) and "Japanese Behçet’s disease Research Committee"(3) that was also stated by Eguia A et al on page E8, right collumn, second paragraph) were reported not only by Adamantiades in 1930, but also from the date back to 18th century in 1772 by Janin(9) as well as by Reis,(10) Blüthe,(11) Gilbert,(12-14) Planner and Remenowsky,(15) Weve(16) and Shigeta(17) (see please simply the related titles of the papers). In the same manner, recurrent oro-mucocutaneous symptoms or findings (still one of the major criteria in both classifications(2,3) that were numbered as 1, 2 and 3 subtitles by Eguia A et al. on page E8, left collumn, clinical features), were not only reported by Adamantiades in 1930, but also from the date back to 1895 by Neumann(18) as well as by Christlieb,(19) Reis,(10) Blüthe,(11) Planner and Remenowsky,(15) Weve,(16) Shigeta,(17) Pils,(20) Grütz,(21) Carol and Ruys,(22) Samek and Fischer,(23) Walter and Roman,(24) and Kumer.(25) Moreover, erythema nodosum (one of the most frequently encountered skin lesion of the disease that was also stated by Eguia A et al. on page E8, right collumn, L3) was reported by Kumer(25) in 1930 whereas neurological signs (a minor criterion of Japanese Behçet’s disease Research Committee(3) that was stated by Eguia A et al. on page E8, right collumn, number 6) were reported not only by Adamantiades in 1930, but also by Weve,(16) Blüthe(11) and Shigeta.(17) Moreover, arthritis and/or orchitis (two minor criteria of Japanese Behçet’s disease Research Committee(3) that was stated by the by Eguia A et al on page E8, right collumn, number 5) was not reported only by Adamantiades in 1930, but also reported clearly by Reis,(10) Blüthe,(11) Weve,(16) Shigeta,(17) and Carol and Ruys.(22) Furthermore, the pathergy test (a major criteria of International Study Group(2) at present that was stated in discussion section by Eguia A et al on E9) was first used by Samek and Fisher(23) in 1929 (before Dr. Adamantiades).

Having presented the chronological evidences on the major and/or minor findings of Behçet disease reported by a number of authors before Dr. Adamantiades and after Hippocrates, let’s explain now why the disease had gained worldwide appreciation in today’s modern medicine as "Behçet disease" or "Behçet syndrome". Dr. Hulusi Behçet was the first physician who recognized a typical set of dermatologic, ocular and oro–genital lesions, did group all these findings himself into a single disease and then published the results in 1937 as an association between 3 unrelated symptoms and signs (Behçet H. Über rezidivierende, Aphthöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien. Dermatol Wochenschr 1937;105:1152–1157), and then in 1939 in German as "Tri–Symptomenkomplex" (Behçet H. Einige Bemerkungen zu meinen Beobachtungen über den Tri–Symptomenkomplex. Med Welt 1939;13:1222–1227) and in 1940 in English as "Triple symptom complex" (Behçet H. Some observations on the clinical picture of the so–called triple symptom complex. Dermatologica 1940;81:73–83), indicating a novel and a separate disease. These publications were supported by following papers from Jensen(26) (who first used the term "Behçet syndrome"), Ephraim(27) ("Triple symptom complex" was secondly used) and Feigenbaum(28) (the term "Behçet disease" was first used). Afterwards in 1947, international dermatological society in Zurich named the disease as "Morbus Behçet" that honored the first describer of "triple synptom complex", not individual symptoms.

A simple PubMed search revealed more than 5810 international published articles and ten–thousands of citations that named the disease as "Behçet disease" or "Behçet syndrome" in their titles in the last 65 years. More importantly, classical Textbooks of Dermatology, Rheumatology, Ophthalmology and any other medical books as well as international congresses and symposiums call this disease again as Behçet disease, not as Adamantiades–Behçet disease. On the other hand, there are only 88 articles that call the disease as "Adamantiades-Behçet".

In direct contradiction of Eguia et al., Dr. Adamantiades himself named the disease as "Behçet" in the title of his one subsequent paper published just 12 years after the original articles of Dr. Hulusi Behçet (Adamantiades, B. and Lorando, N. Sur le syndrome complexe de uvéite récidivante ou soi–distant syndrome complexe de Behçet. Presse Med 1949;57:501).

Therefore, I think it is not the author’s intention to personally change a name of an internationally accepted disorder by reporting some individual indications, which is not in accordance with the whole history of the disease stated up to now. Authors must know historical realities deeper and obey themselves to the knowledge of positive sciences. International Behçet’s Society clearly acknowledges the origin of Behçet disease in his official web site and strongly proposes the eponym "Behçet", not "Adamantiades–Behçet.(6-8) Furthermore, both "American Behçet’s Disease Association"(29) and "Japanese Behçet's disease Research Committee"(3) call this disease again as "Behçet disease". Authors must follow the instructions of International Groups,(2) Societies,(7) Associations(29) and Research Committees strictly.(3) Otherwise, we, as researchers, can change every name of the disease with some personal indications that would be ended with naming dilemma and confusion among the auhtors, patients and the editors of the respected journals.


Cem Evereklioglu, MD
Department of Ophthalmology
Erciyes University Medical Faculty
Kayseri – Turkey



1. Evereklioglu C. Current concepts in the etiology and treatment of Behçet diseae (Major Review). Surv Ophthalmol 2005;50:277–350.

2. International Study Group for Behçet's Disease: Criteria for diagnosis of Behçet's disease. Lancet 1991;335:1078–80.

3. The Behçet's Disease Research Committee of Japan. Skin hypersensitivity to streptococcal antigens and the induction of systemic symptoms by the antigens in Behçet’s disease: a multicenter study. J Rheumatol 1989;16:506–11.

4. Adamantiades B. A case of recurrent hypopyon iritis. Proceedings of The Medical Society of Athens 1930;586–593.

5. Dils en N. History and development of Behçet's disease. Rev Rhum Engl Ed 1996;63:512–9.

6. Giannoukas AD. Regarding "compelling nature of arterial manifestations in Behçet disease" (Letter to the editor and author reply). J Vasc Surg 2005;41:181–2.

7. Behçet’s Syndrome Society. http://www.Behç (Accessed April 26, 2006).

8. Evereklioglu C. Author's response. Surv Ophthalmol 2006;51:174-7.

9. Janin J. (1772) Mémoires et observations anatomiques, physiologiques et physiques sur l’œil, et sur les maladies qui affectent cet organe. Avec un précis des opérations et des remédies qu'on doit pratiquer pour les guerres. Frères Périsse, Lyon–PF Didot, Paris, pp 412–4.

10. Reis W. Augenerkrankung und Erythema nodosum. Klin Monatsbl Augenheilkd 1906;44:203–6.

11. Blüthe L. Zur Kenntnis des rezidivierenden Hypopyons. Inauguralthesis, D Strauss, Heidelberg 1908.

12. Gilbert W. Über die rezidivierende eitrige Iridozyklitis (I. septica) und ihre Beziehungen zur septischen Allgemeinerkrankung. Arch Augenheilkd 1920;86:29–49.

13. Gilbert W. Über den pathologisch–anatomischen Befund bei Iridocyclitis septica (Iritis mit rezidivierendem Hypopyon). Arch Augenheilkd 1921;87:27–34.

14. Gilbert W. Zur Frage der Iridozyklitis mit rezidivierendem Hypopyon ("Iritis septica"). Klin Monatsbl Augenheilkd 1923;71:409–14.

15. Planner H, Remenowsky F. Beiträge zur Kenntnis der Ulcerationen am außeren weiblichen Genitale. Arch Dermatol Syphil (Berlin) 1922;140:162–88.

16. Weve H. Über rezidivierende allergische Staphylokokkenuveitis. Arch Augenheilkd 1923;93:14–39.

17. Shigeta T. Recurrent iritis with hypopyon and its pathological findings. Acta Soc OphthalmoI Jpn 1924;28:516–22.

18. Neumann I. Die Aphthen am weiblichen Genitale. Wien Klein Rundsch 1895;9:289–307.

19. Christlieb O. Über Stomatitis und Vulvitis aphthosa. Inaugural–Dissertation. Universität Würzburg 1895.

20. Pils H. Ein Beitrag zur Aphthosis. Arch Dermatol Syphil (Berlin) 1925;149:4–8.

21. Grütz O. Stomatitis et vulvitis aphthosa chronica rezidivans (blastomycetica)? Zbl Haut 1926;20:415–6.

22. Carol WL, Ruys SC. Over aphthosis en ulcus vulvae acutum. Ned Tschr Genek 1928;1:396–406.

23. Samek J, Fischer E. Erythema nodosum als bakterielle Metastase eines Ulcus vulvae acutum. Arch Dermatol Syphil (Berlin) 1929;158:729–33.

24. Walter F, Roman I. Beitrag zur Kenntnis der hämatogenen Hautmetastasen bei Ulcus vulvae acutum. Dermatol Wochenschr 1930;90:705–9.

25. Kumer L. Über Haut–und Mundschleimhauterscheinungen beim Ulcus vulvae acutum. Dermatol Z 1930;57 :401–11.

26. Jensen T. Sur les ulcérations aphteuses de la muqueuse de la bouche et de la peau génitale combinées avec les symptômes oculaires (=Syndrome Behçet). Acta Dermatol Venereol 1941;22:64–79.

27. Ephraim H. Triple symptom complex of Behçet. Arch Dermatol Syphil (Chicago) 1944;50:37–8.

28. Feigenbaum A, Kornblueth W. Behçet’s disease as manifestation of a chronic septic condition connected with a constitutional disorder. With a report of 4 cases. Acta Med Orient 1946;5:139–51.

29. http://www.Behç & b=260523 (Accessed April 26, 2006).




Dear Editor,

We have read the letter submitted by Dr. Evereklioglu related to our revision work entitled: "Adamantiades-Behçet disease: an enigmatic process with oral manifestations. Med Oral Patol Oral Cir Bucal. 2006 ; 11: E6-1".

Unfortunately, we aren’t "Behçetologists" or "adamantiadologists"; we are just specialist in Oral Pathology who is interested in this disease and in the patients that suffer from it.

We do appreciate the in-depth information provided by Dr. Evereklioglu in his letter, which completes in a great manner the historical aspects of this fascinating pathology. Maybe, really, the correct name would be "Hippocrates disease". Nevertheless, we would have a huge problem if we have to apply this rule to all the diseases described by Hippocrates.

In Medicine there are a lot of examples were there have been names which have been left behind: Albright syndrome (McCune-Albright), Gorlin syndrome (Gorlin-Goltz), Sjögren syndrome (Gougerot-Sjögren), etc…

We leave the debate about the terminology of the disease to experts such as Dr. Evereklioglu. Our intention was, according to the antecedents (more of 80 papers in PUBMED), to try to widen the spectrum of the denomination.

Sincerely yours,


Asier Eguia, José Manuel Aguirre
Medicina Bucal
Unidad de Patología Oral y Maxilofacial
Facultad de Medicina y Odontología.
Universidad del País Vasco EHU

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