International Ophthalmology

, Volume 27, Issue 2–3, pp 67–79 | Cite as

Vogt–Koyanagi–Harada disease: inquiry into the genesis of a disease name in the historical context of Switzerland and Japan

  • Carl P. Herbort
  • Manabu Mochizuki
Original Paper



To delineate the historical steps associated with the genesis of the name and the definition of Vogt–Koyanagi–Harada (VKH) disease.


A bibliographical review of the major publications that were relevant to the original development of the name of the clinical entity known today as Vogt–Koyanagi–Harada disease, in the historical context of the early 20th century.


Three distinct time periods can be considered to be important in terms of providing a historical perspective on VKH disease. Given that the cutaneous manifestations of VKH disease are so characteristic, these could not have been missed even before the actual clinical entity of VKH was recognized in the early 20th century. Indeed, several authors, including the Arabic doctor Mohammad-al-Ghâfiqî in the 12th century as well as Jacobi, Nettelship and Tay in the 19th century, described poliosis, neuralgias and hearing disorders. Many of these cases were probably due to sympathetic ophthalmia, but some were clearly VKH cases. The second phase is characterized by the surge of articles that appeared early in the 20th century that defined the disease more precisely. A number of these authors subsequently became associated with the disease name, the first being Alfred Vogt from Switzerland, followed by Japanese researchers. Yoshizo Koyanagi was in fact not the first Japanese author to describe the disease; this honor goes to the first Japanese Professor of Ophthalmology at the University of Tokyo, Dr. Jujiro Komoto, who published in a German language journal, Klinische Monatsblätter für Augenheilkunde in 1911. Yoshizo Koyanagi published his first report in the Nippon Ganka Gakkai Zasshi 3 years later, in 1914, but it was a much later article, one published in 1929, that definitively associated his name with the disease. In this review article, Koyanagi reported 16 cases, of which six were his own cases, that beautifully illustrate the natural course of the disease. In this same time period, Einosuke Harada, in an article published in Nippon Ganka Gakkai Zasshi in 1926 that was based on several case studies, comprehensively described a syndrome that included (1) a prodromal phase of malaise and meningeal irritation; (2) bilateral uveitis of diverse intensity; (3) bilateral retinal detachments spontaneously resolving; (4) integumentary changes; (5) lymphocytosis of the spinal fluid; (6) dysacousia. It is now accepted that Vogt–Koyanagi disease and the syndrome described by Harada are one entity with a diverse clinical spectrum bearing the universally accepted name of Vogt–Koyanagi–Harada disease. The third phase and most recent phase is characterized by the rapid progress made in terms of knowledge of the physiopathology of the disease, primarily due to the development of immunological methods. The evidence accumulated to date clearly points towards an autoimmune Th1 disease directed against proteins associated with choroidal melanin. Other analytical techniques, such as indocyanine green angiography, have enabled researchers to monitor more closely the primary lesional process at the level of the choroid, and standardized diagnostic criteria have been generated in the recent past.


Those who earn scientific merit in clinical medicine are the ones who are able to visualize an overview based on the synthesis of ‘new' medical facts that have been made available, usually reported singly by several, unassociated authors concomitantly. This is certainly the case for Yoshizo Koyanagi and Einosuke Harada. Conversely, Alfred Vogt was primarily lucky in that he encountered and subsequently precisely described the first case in the literature.


History Japan Switzerland Vogt–Koyanagi–Harada disease 



Both authors would like to express their gratitude to the late Professor Saichi Mishima, who was one of their teachers. His monumental work, carried out with a team of Japanese doctors, on the history of ophthalmology in Japan was used extensively for the ophthalmic historical part of this work. Dr. Herbort would like to thank Dr. Luca Cimino in Italy who encouraged him to undertake this work. Dr. Cimino carried out a literature search, forwarded the original articles and convinced him that he (CPH) was the person predestined to write about the European aspect of VKH as Vogt was Swiss and as the main articles were written either in German, even for the Japanese authors, or in French (for the article that first linked the two names of Vogt and Koyanagi). Thanks are extended from Dr. Herbort to the whole staff of the Department of Ophthalmology and Visual Science of Tokyo Medical and Dental University who warmly welcomed him in their midst and allowed him to plunge into the reality of VKH disease. Thanks also are directed from Dr. Herbort to his wife Naoko who assisted him in this work, critically reviewing its historical part and being very helpful in the translation of some Japanese texts.


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Copyright information

© Springer Science+Business Media, Inc. 2007

Authors and Affiliations

  1. 1.Inflammatory and Retinal Eye DiseasesCentre for Ophthalmic Specialized Care (COS)LausanneSwitzerland
  2. 2.Foundation Memorial A. de RothschildGenevaSwitzerland
  3. 3.University of LausanneLausanneSwitzerland
  4. 4.Department of Ophthalmology and Visual ScienceTokyo Medical and Dental University Graduate SchoolTokyoJapan

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