Hulûsi Behçet

Biography

• Born February 20, 1889, Istanbul, Turkey
• 1906-1910 Completed his medical qualification (aged 21 years) as a military doctor at Gülhane Military Medical Academy School of Medicine, Istanbul, Turkey
• 1910-1914 Resident in dermatology and venereal diseases at Gülhane Military Medical Academy Hospital dermatology clinic
• 1914 – Assistant chief physician of Kirklareli Military Hospital
• 1914-1918 (WW1) Worked as a dermatovenereologist at the Edirne Military Hospital, Turkey
• 1919 – Further medical training in Budapest and then Charité hospital, Berlin
• 1923 – Director physician at the Hasköy Venereal Diseases Hospital – Shortly later, worked as a dermatologist at Guraba Hospital (now Istanbul Medical College)
• 1933-1947 Professor and director of the Department of Dermatology and Venereology at the University of Istanbul, Turkey
• 1937 – Described two cases with the triad associated with Behçet disease of aphthous mouth ulcers, genital ulcers, and recurrent iritis, and proposed this as a distinct disease entity in the journal Deri Hastaliklari ve Frengi Arsivi [Turkish Archives of Dermatology and Syphilology], then internationally in the German journal Dermatologische Wochenschrift (1937).
• 1939 – Named the disease in his German publication as ‘tri symptomen komplex’, then in English as the ‘triple symptoms complex’ in 1940
• 1939 – Awarded the title of Ordinarius Professor for his lifetime achievements from the University of Istanbul
• Died 8 March 1948, Istanbul, Turkey

Medical Eponyms

Behçet disease (1937)

Chronic, multisystem inflammatory condition involving small and large vessels of unknown aetiology. It is characterised by the classical triad of recurrent oral aphthous ulcers, genital ulcers, and iridocyclitis with or without hypopyon.

1937 – Behçet published two cases with the clinical triad of lesions in the mouth, genitalia, conjunctivae, and inflammation of the iris, which were either simultaneous or periodic, and relapsed several times a year. He postulated a new systemic disease of viral aetiology after investigation for numerous differential diagnoses, such as tuberculosis, syphilis, diphtheria, simple ulcers, Nicolas-Favre syndrome, and several other dermatological conditions, were negative.

• Behçet Case 1: Otherwise healthy 40 year-old man with ulceration of his mouth, scrotum and cornea (traumatic); and perikeratitis and iritis over the preceding 20 years. Other physicians who consulted the patient prior thought the manifestations were of tuberculosis, syphilis, or parasites, and treatment with gold and arsenic seemed to reduce relapses initially. The patient underwent several iridectomies, but eventually became blind.

• Behçet Case 2: 34 year-old woman, otherwise well with healthy children, who had a seven year history of relapsing painful lesions starting on the tongue then spreading throughout the mouth, and eventually involving the genitalia (vulva). Corneal erosions and episcleritis were also observed. Each relapse lasted up to one month. Biopsies of the orogenital ulcers on microscopy showed chronic inflammation, vessel wall thickening and elementary viral particles that was negative on subsequent biopsies.

The cases which are considered here exhibit the following clinical picture: lesions in the mouth, on the genitalia, and of the eyes…the lesions of the relapses which occurred several times a year appeared either simultaneously or successively in the mouth, on the genitalia, on the conjunctiva, and even as an inflammation of the iris

Behçet 1937

• Behçet H. Über rezidivierende, aphtöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien [On relapsing, aphthous ulcers of the mouth, eye and genitalia caused by a virus]. Dermatologische Wochenschrift. 1937;105:1152–63.

1938 – Behçet published another case of a male patient with oral pemphigus-like lesions, scrotal ulcers, eye irritation, acneiform lesions on the back, fevers, abdominal pain, and a dental cyst.

• Behçet H. Considerations sur les lesions aphtheuses de la bouche et des parties genitals, ainsi que sur les manifestations oculaires d’origine probablement virutique et observations concernant leur foyer d’infection. Bull Soc Fr Dermatol Syphiligr. 1938;45:420–433.

1938-40 – Behçet published four additional cases separately in German, French and English with what he named the ‘triple symptom complex’. He observed additional characteristics of acneiform skin lesions, erythema nodosum, periodontitis, and arthralgia as part of what he argued was a new disease of potential viral aetiology. In his 1940 English publication, Behçet referred to the cases reported by Adamantiades (1931), Wewe (1923), and Blobner (1937) whom he stated considered the ocular inflammation separately and not as another manifestation along with skin and mucocutaneous symptoms.

• Behçet H. Kurze Mitteilung über Fokalsepsis mit aphthoÈsen Erscheinungen an Mund, Genitalien und VeraÈnderungen an den Augen, als wahrscheinliche Folge einer durch Virus bedingten Allgemeininfektion. Dermatol Wochenschr. 1938;107:1037-40.
• Behçet H. A propos d’une entité morbide due probablement à un virus spécial donnant lieu à une affection géné ralisée se manifestant par poussées récidivantes en trois régions principales et occasionnant en particulier des iritis répétés. Bull Soc Fr Dermatol Syph. 1939;46:674-687.
• Behçet H. Einige Bemerkungen zu meinen Beobachtungen über den Tri Symptomen komplex. Med Welt. 1939;13:1222-27.
• Behçet H. Some observations on the clinical picture of the so-called triple symptom complex. Dermatologica. 1940;81:73–78.

Key Medical Attributions

He also had a strong interest in the clinical, skin manifestations, epidemiological, and social aspects of syphilis, publishing a comprehensive book on the disease in 1940 titled ‘Clinical and Practical Syphilis, Diagnosis and Related Dermatoses’. Published one of the first Turkish journals in dermatology: ‘Turkish Archives of Dermatology and Syphilology’.

Behçet’s other areas of research included:

• Cutaneous leishmaniasis (Leishmaniasis cutis), which he provided the first description of the pathognomonic ‘nail sign’ associated with the parasitic infection. The ‘nail sign’ describes nail-like projections from the crust that develops under an ulcerated nodule in the infection.
• Treatments of superficial and deep mycosis – Dermatitis figus carica, an allergic dermatosis that develops after contact with figs and their leaves.
• Properties of ‘gale cereal’ and barley in protecting workers against common parasitic infections from the Black Sea.

Major Publications

• Behçet H. Über rezidivierende, aphtöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien [On relapsing, aphthous ulcers of the mouth, eye and genitalia caused by a virus]. Dermatologische Wochenschrift. 1937;105:1152–63. [Translation: Behçet H, Matteson EL. On relapsing, aphthous ulcers of the mouth, eye and genitalia caused by a virus. 1937. Clin Exp Rheumatol. 2010;28(4 Suppl 60):S2-5.
• Behçet H. Considerations sur les lesions aphtheuses de la bouche et des parties genitals, ainsi que sur les manifestations oculaires d’origine probablement virutique et observations concernant leur foyer d’infection. Bull Soc Fr Dermatol Syphiligr. 1938; 45: 420–433.
• Behçet H. Kurze mitteilung über fokal septis mit aphtösen erscheinungen an mund, genitalen und veranderungen an den augen, als wahrscheinliche folge einer durch virusbedingten allgemeininfection. Dermatologische Wochenschrift. 1938;1 07: 1037-40.
• Behçet H. A propos d’une entité morbide due probablement à un virus spécial donnant lieu à une affection géné ralisée se manifestant par poussées récidivantes en trois régions principales et occasionnant en particulier des iritis répétés. Bull Soc Fr Dermatol Syph. 1939; 46: 674-687.
• Behçet H. Einige Bemerkungen zu meinen Beobachtungen über den Tri Symptomen komplex. Med Welt. 1939;13:1222-27.
• Behçet H. Some observations on the clinical picture of the so-called triple symptom complex. Dermatologica. 1940;81:73–78
• Behçet H. Klinikte ve Pratikte Frengi Teşhisi ve Benzeri Deri Hastalıkları [Clinical and Practical Syphilis, Diagnosis and Related Dermatoses]. 450 sh. (ayrıca 130 sh. Resim), Istanbul, Kenan Basımevi, 1940.

Controversies

His description of Behçet disease as a new entity was initially rejected by the majority of dermatologists, who argued that the symptoms reflected other conditions, such as pemphigus, ulcus vulvae acutum, dermatomyositis, Neumann atrophy, and erythema exudativum multiforme.

References

Biography

• Saylan T. Life story of Dr. Hulusi Behçet. Yonsei Med J. 1997; 38(6): 327-332.
• UStün C. A famous Turkish dermatologist, Dr. Hulusi Behçet. Eur J Dermatol. 2002 Sep-Oct;12(5):469-70
• Tüzün Y. Hulusi Behçet, MD: February 20, 1889 to March 8, 1948. Clin Dermatol. 2006 Nov-Dec;24(6):548-50.
• Tan SY, Poole PS. Hulusi Behçet (1889–1948): Passion for dermatology. Singapore Med J. 2016 Jul;57(7):408-9.
• Shakeri A. Hulusi Behçet—A Life of Passion and Endeavor in Dermatology. JAMA Dermatol. 2018; 154(4):440-440
• Prof. Dr. Hulusi Behçet 1889-1948. hulusibehcet.net
• Baskett TF. Behçet, Hulusi (1889–1948) In: Eponyms and Names in Obstetrics and Gynaecology. 3e. 2019
• Bibliography. Behçet, Hulûsi 1889-1948. WorldCat Identities

Eponymous term

• Zouboulis CC, Keitel W. A historical review of early descriptions of Adamantiades-Behçet’s disease. J Invest Dermatol. 2002 Jul;119(1):201-5
• Evereklioglu C. Regarding neutrophil and lymphocyte responses to oral Streptococcus in Adamantiades-Behçet’s disease. FEMS Immunol Med Microbiol. 2006 Aug;47(3):311-4.