Report
The prevalence of Behc ß et’s disease in a city in Central Anatolia in Turkey 2 € €lgecßen1, MD, Kemal Ozyurt Emine C , MD, Ayten Ferahbasß3, MD, Murat Borlu3, MD, ßo 6 € urk5, PhD, Aysße Ozt€ € urk Oner € _ , MD, Iskender G€ un7, MD, Pınar Kulluk4, MD, Ahmet Ozt€ 3 € and Ozcan Asßßcıo glu , MD
1 Department of Dermatology, Faculty of Medicine, Bozok University, Yozgat, Turkey, 2Department of Dermatology, _ € Imam €tcßu Faculty of Medicine, Su University, Kahramanmarasß, Turkey, 3 Department of Dermatology, Faculty of Medicine, Erciyes University, Kayseri, Turkey, 4Department of Dermatology, Erdem Hospital, Istanbul, Turkey, Departments of 5Biostatistics, 6 Ophthalmology, and 7 Public Health, Faculty of Medicine, Erciyes University, Kayseri, Turkey
Correspondence €lgecßen, MD Emine C ßo Department of Dermatology Bozok University Medical Faculty TR-66000 Yozgat, Turkey E-mail: [email protected] Conflicts of interest: None. Funding: Erciyes University Research Fund (project no. TT-05-24).
Abstract Background The prevalence of Behcßet’s disease (BD) is much higher in countries along the ancient Silk Route, extending from Japan to Mediterranean countries including Turkey, than in northern Europe and the USA. Objectives The present study aimed to investigate the prevalence of BD in Kayseri, a city in Central Anatolia in Turkey. Methods This study investigated cross-sectional prevalences of BD in individuals aged >10 years in Kayseri, Turkey, in two stages. The first stage aimed to identify individuals with recurrent oral ulcers (ROUs) through home visits, and the second stage aimed to further examine those with ROUs for the presence of other BD-related manifestations under hospital conditions. The study was conducted using the criteria defined by the International Study Group for Behcßet’s Disease. The sample size was determined to be 4697 with an expected sampling error of 5.5 per 10,000, with a 95% confidence interval. A standard questionnaire was administered to a total of 5218 individuals. Results A history of ROU was recorded in 470 (9.0%) of the 5218 residents, and a previous diagnosis of BD was recorded in nine individuals. The prevalence rate of BD was estimated as 17 cases per 10,000 population in Kayseri, Turkey. Conclusions The present study contributes towards estimations of prevalences of BD in Turkey and towards raising public awareness about the disease. It also supports previous studies that have reported the world’s highest prevalences of BD in Turkey.
Introduction
286
Behcßet’s disease (BD) was first described by Hulusi Behcßet in 1937 in three patients with a triple symptom complex of aphthae, genital ulcers, and hypopyon uveitis.1 It is a chronic multisystem disorder characterized by recurring inflammatory processes.2,3 Although BD has been reported all over the world, it has a distinct geographic distribution. Reported rates show regional differences within and between countries. It occurs most frequently between the latitudes of 30 °N and 45 °N in Asian and European populations; geographically this suggests a possible relationship with the historic silk trading route. Large epidemiological surveys are lacking, and prevalence figures in many countries are based on case registries and hospital attendance.4,5 In Turkey, five different surveys6–10 have reported prevalence rates in International Journal of Dermatology 2015, 54, 286–289
the range of 20–421 per 100,000 population, according to which Turkey is ranked as the country with the highest rate of BD in the world. However, these surveys6–10 were carried out in different regions of Turkey and screened selected rural areas or urban areas (Table 1). A nationwide, population-based study establishing the prevalence of BD in Turkey is lacking. The present study was conducted to aid in estimating the prevalence rate of BD in Turkey and to raise public awareness of the disease. Materials and methods Study design
This study investigated cross-sectional prevalences of BD in individuals aged >10 years in Kayseri, a city located in the center of Anatolia. The study was carried out in two ª 2014 The International Society of Dermatology
C ß o€ lgecß en et al.
Prevalence of Behc ß et’s disease in a Turkish city
Report
Table 1 Field surveys of Behc ßet’s disease (BD) conducted in Turkey
Year, authors 1981, 1988, 2002, 2003, 2004,
Demirhindi et al.6 Yurdakul et al.7 Idil et al.8 Azizlerli et al.9 Cakir et al.10
Region, city, all field surveys
BD patients, n
Patients aged >10 years, n
Prevalence per 100,000 population
Istanbul, rural (Northeast), Ordu, rural Ankara, suburban Istanbul, urban (Thrace), Edirne, rural
4 19 16 101 1
4960 5131 13 894 23 986 (>12 years) 4861
80 370 115 421 20
stages. The first stage identified individuals with recurrent oral ulcers (ROUs) through home visits; the second stage further examined those with ROUs for the presence of other BD-related manifestations under hospital conditions. The first stage of the study
The population of Kayseri was reported to be 1,060,432 in the Turkish census carried out in 2000. The sample size was determined to be 4697 with an expected sampling error of 5.5 per 10,000, with a 95% confidence interval (CI). A total of 5218 individuals aged >10 years and living in the city center and the districts of Kayseri were included in this study. The areas covered by 30 primary healthcare centers in the city center and in 14 districts of Kayseri were selected to represent the study site using a simple random sampling method. Individuals to be included in this study were identified using a systematic sampling method from the medical records of the primary healthcare centers selected. The number of individuals to be screened in each primary healthcare center area was determined in proportion to the population covered by all primary healthcare centers in Kayseri. The 5218 individuals were interviewed using a standard questionnaire administered by dermatologists. The standard questionnaire used in this study was prepared mainly for the purposes of identifying individuals with ROUs or those with a diagnosis of BD. All individuals with ROUs and those who had been diagnosed previously with BD elsewhere were invited to attend the BD Outpatient Clinic at the Department of Dermatology, Erciyes University, Kayseri, for further investigation.
In this study, BD was diagnosed according to the criteria defined by the International Study Group for Behcßets Disease (ISGBD)11 (Table 2). Results The study was comprised 5218 residents aged from 10 years to 83 years. The mean standard deviation (SD) age was 33.8 14.7 years. A history of ROUs was recorded in 470 (9.0%) participants, and a previous diagnosis of BD was recorded in nine individuals. In the second stage of the study, 442 of the 470 (94.0%) ROU patients were confirmed as having true ROUs; the remainder were found to have other oral lesions, such as herpes simplex stomatitis, mucosal ulceration caused by dental trauma, scrotal tongue, oral lichen planus, and pemphigus vulgaris. Individuals with true ROUs were examined for other symptoms of BD (genital ulcers, erythema nodosum, acneiform lesions, folliculitis,
Study Group for Behcßet’s Disease diagnostic (classification) criteria11 (1990)a
Table 2 International
Recurrent oral ulceration Plus two of: Recurrent genital ulceration Eye lesions
Second stage of the study
In this stage of the study, individuals who had indicated the presence of ROUs or BD during the first stage of the study were examined for other BD-related manifestations. This investigation included a physical examination and skin pathergy test. All individuals with ROUs and one of the other BD-related manifestations underwent a complete ophthalmological examination by a ophthalmologist at the Uveitis Outpatient Clinic, Department of Ophthalmology. ª 2014 The International Society of Dermatology
Skin lesions
Pathergy
Minor aphthous, major aphthous, or herpetiform ulceration observed by the physician or patient and recurring at least three times in one 12-month period
Aphthous ulceration or scarring, observed by the physician or patient Anterior uveitis, posterior uveitis, cells in the vitreous on slit-lamp examination, or retinal vasculitis observed by the ophthalmologist Erythema nodosum observed by the physician or patient, pseudofolliculitis, papulopustular lesions or acneiform nodules observed by the physician in post-adolescent patients not on corticosteroid treatment Read by the physician at 24–48 h
a
Findings are applicable only in the absence of other clinical explanations. International Journal of Dermatology 2015, 54, 286–289
287
288
Report
C ß o€ lgecß en et al.
Prevalence of Behc ß et’s disease in a Turkish city
arthritis). Of the 442 patients with true ROUs, pathergy tests were performed in 420 (95.0%). Of these 420 patients, results were negative in 328 (78.1%) patients. Ophthalmological examinations were performed in 42 patients with ROUs and one of the other BD-related manifestations. A diagnosis of BD according to the ISGBD criteria11 was established in nine individuals. All nine of these patients had been previously diagnosed with BD and had been followed up at various centers. Of these nine patients, four (44.4%) were female and five (55.6%) were male. Their ages ranged between 27 years and 58 years (mean SD age: 40.9 10.4 years). Clinical findings in the nine patients are summarized in Table 3. Recurrent oral ulcers were observed in all of the nine patients, genital ulcers in eight, ocular involvement in three, arthritis in one, and arthralgia in five. Skin manifestations in these nine patients included pseudofolliculitis in six patients, erythema nodosum in four patients, and extragenital ulcers in one patient. The skin pathergy test was positive in four (44.4%) of the nine patients. The prevalence of mainly mucocutaneous involvement was 44.4%, whereas the prevalence of vital organ involvement was 55.6% (five patients, including three male and two female patients, of whom three had ocular involvement and two had deep venous thrombosis). A family history of BD was recorded in four (44.4%) of the nine patients. In conclusion, the prevalence of BD in Kayseri, Turkey, was calculated as 9/5218 = 0.001725 and thus as 17 cases per 10,000 population (95% CI 8–33) in this survey. Discussion Behcßet’s disease is common in those countries that extend from the Mediterranean to the Far East along the ancient trading route known as the Silk Road. A nationwide Table 3 Clinical features in patients diagnosed with Behc ßets
disease
Recurrent oral ulcers Genital ulcer Eye involvement Pseudofolliculitis Erythema nodosum Arthritis Arthralgia Deep vein thrombosis Extragenital ulcers Positive pathergy test
n (F, M)
%
9 8 3 6 4 1 5 2 1 4
100 88.9 33.3 66.6 44.4 11.1 55.5 22.2 11.1 44.4
(4, (3, (1, (3, (3, (0, (2, (1, (0, (1,
5) 5) 2) 3) 1) 1) 3) 1) 1) 3)
F, female; M, male. International Journal of Dermatology 2015, 54, 286–289
survey based on hospital records revealed an estimated prevalence of BD in the Japanese population of 7–8.5 cases per 100,000 population.1–3 One field survey showed a prevalence of 17 cases per 100,000 population in Iraq, and another reported a frequency of 120 cases per 100,000 population in an Arab community in Israel.12,13 These frequencies are distinctly higher than that found among ethnic Jews.12,13 Estimated prevalences elsewhere range from 0.64 per 100,000 population in the UK and 6.4 per 100,000 population in Spain, to 8.6 per 100,000 population in the USA.3 Five previous studies have focused on the frequency of BD in Turkey; reported prevalences ranged from 20 to 421 per 100,000 population.6–10 Prevalences of BD were found to be 80 per 100,000 in Silivri near Istanbul,6 370 per 100,000 in C ß amasß in northern Anatolia,7 110 per 100,000 in the Park region of Ankara,8 421 per 100,000 in Istanbul,9 and 20 per 100,000 in a rural population in Havsa.10 Apart from the differences in prevalence, differences in the frequencies of various manifestations of BD were also identified in these studies. No individuals with ocular involvement were identified in C ß amasß and Havsa,7,10 but the frequency of ocular involvement was reported to be 50.0% in Silivri,6 43.7% in the Park region of Ankara,8 27.7% in Istanbul,9 and 33.3% in the present study. The frequency of a positive pathergy test was 33.3% in Silivri and C ß amasß,6,7 81.2% in the Park 8 region of Ankara, 69.3% in Istanbul,9 and 44.4% in this study. Findings of a male predominance in patients with a severe course of disease are consistent with previous reports.2,3,9 The varying prevalence rates of BD in Turkey (20–421 per 100,000 population) may relate to regional differences, different sample sizes, and particular urban and rural characteristics. The present study included both rural and urban areas, but three of the areas studied previously (C ß amasß, Silivri, Havsa) were rural, and the other two (Ankara, Istanbul) were suburban or urban. Istanbul is a large cosmopolitan city with very high population motility. This phenomenon might contribute to increases in the prevalence of BD, as well as of other diseases. The populations of C ß amasß, Ankara, and Kayseri largely consist of Anatolian Turks, whereas the population of Havsa is more homogeneous and includes a higher proportion of Balkan Turks. The lower prevalence identified in Havsa may relate to the ethnic distribution of its population. Although ROUs represent the symptom most frequently seen in BD, in the present study only nine of 442 individuals with ROUs (20 per 1000 population) were found to have BD. Findings in the other studies reported from Turkey varied. In C ß amasß, 19 of 417 individuals with ROUs (45 per 1000 population) had BD.7 In Ankara, 16 ª 2014 The International Society of Dermatology
C ß o€ lgecß en et al.
of 823 individuals with ROUs (19 per 1000 population) had BD.8 In Istanbul, 101 of 700 individuals with ROUs (144 per 1000 population) had BD,9 and in Havsa, one of 115 individuals with ROUs (9 per 1000 population) had BD.10 In a study from South Korea, Bang and colleagues observed that 50% of patients with ROUs developed BD.14 However, unlike the Turkish studies,6–10 which were based on field surveys, the study reported by Bang et al.14 was based on hospital records. Thus disparities between the results of studies from the two countries may be attributable to dissimilarities in their methods, and to ethnic, genetic, and geographical differences. In conclusion, the prevalence of BD varies among different countries. In Turkey, a nationwide, populationbased study investigating the prevalence of BD is lacking. The fact that this study found no new cases of BD may be explained by the increased healthcare facilities available in Kayseri. The present study will contribute towards the estimation of prevalence rates of BD in Turkey and will help to raise public awareness of this disease. Its findings also support those of previous studies that have reported Turkey as having the world’s highest prevalence of BD6–10. References 1 Behcßet H. Uber rezidivierende, aphthose, durch ein Virus verursachte Geshwure am Munde, am Auge und an den Genitalien. Dermatol Wochenschr 1937; 36: 1152–1157. 2 Sakane T, Takeno M, Suzuki N, et al. Behcßets disease. N Engl J Med 1999; 41: 1284–1291. 3 Yurdakul S, Yazici H. Behcßets syndrome. Best Pract Res Clin Rheumatol 2008; 22: 793–809.
ª 2014 The International Society of Dermatology
Prevalence of Behc ß et’s disease in a Turkish city
Report
4 James DG. Silk route disease. Postgrad Med J 1986; 62: 151–153. 5 Verity DH, Marr JE, Ohno S, et al. Behcßets disease, the Silk Road and HLA-B51: historical and geographical perspectives. Tissue Antigens 1999; 54: 213–220. 6 Demirhindi O, Yazıcı H, Binyıldız P, et al. Silivri Fener Koyu ve yoresinde Behcßet Hastalı gı sıklı gı ve bu hastalı gın toplum icinde taranmasında kullanılabilecek bir yontem. Cerrahpasa Tıp Fak Derg 1981; 12: 509–514. 7 Yurdakul S, Gunaydin I, Tuzun Y, et al. The prevalence of Behcßets syndrome in a rural area in northern Turkey. J Rheumatol 1988; 15: 820–822. 8 Idil A, Gurler A, Boyvat A, et al. The prevalence of Behcßets disease above the age of 10 years. The results of a pilot study conducted at the Park Primary Health Care Center in Ankara, Turkey. Ophthalmic Epidemiol 2002; 9: 325–331. 9 Azizlerli G, Kose AA, Sarica R, et al. Prevalence of Behcßets disease in Istanbul, Turkey. Int J Dermatol 2003; 42: 803–806. 10 Cakir N, Dervis E, Benian O, et al. Prevalence of Behcßets disease in rural western Turkey: a preliminary report. Clin Exp Rheumatol 2004; 22: 53–55. 11 International Study Group for Behcßets Disease. Criteria for diagnosis of Behcßets disease. Lancet 1990; 335: 1078–1080. 12 Al-Rawi ZS, Neda AH. Prevalence of Behcßets disease among Iraqis. Adv Exp Med Biol 2003; 528: 37–41. 13 Jaber L, Milo G, Halpern GJ, et al. Prevalence of Behcßets disease in an Arab community in Israel. Ann Rheum Dis 2002; 61: 365–366. 14 Bang D, Yoon KH, Chung HG, et al. Epidemiological and clinical features of Behcßets disease in Korea. Yonsei Med J 1997; 38: 428–436.
International Journal of Dermatology 2015, 54, 286–289
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The prevalence of Behc ß et’s disease in a city in Central Anatolia in Turkey 2 € €lgecßen1, MD, Kemal Ozyurt Emine C , MD, Ayten Ferahbasß3, MD, Murat Borlu3, MD, ßo 6 € urk5, PhD, Aysße Ozt€ € urk Oner € _ , MD, Iskender G€ un7, MD, Pınar Kulluk4, MD, Ahmet Ozt€ 3 € and Ozcan Asßßcıo glu , MD
1 Department of Dermatology, Faculty of Medicine, Bozok University, Yozgat, Turkey, 2Department of Dermatology, _ € Imam €tcßu Faculty of Medicine, Su University, Kahramanmarasß, Turkey, 3 Department of Dermatology, Faculty of Medicine, Erciyes University, Kayseri, Turkey, 4Department of Dermatology, Erdem Hospital, Istanbul, Turkey, Departments of 5Biostatistics, 6 Ophthalmology, and 7 Public Health, Faculty of Medicine, Erciyes University, Kayseri, Turkey
Correspondence €lgecßen, MD Emine C ßo Department of Dermatology Bozok University Medical Faculty TR-66000 Yozgat, Turkey E-mail: [email protected] Conflicts of interest: None. Funding: Erciyes University Research Fund (project no. TT-05-24).
Abstract Background The prevalence of Behcßet’s disease (BD) is much higher in countries along the ancient Silk Route, extending from Japan to Mediterranean countries including Turkey, than in northern Europe and the USA. Objectives The present study aimed to investigate the prevalence of BD in Kayseri, a city in Central Anatolia in Turkey. Methods This study investigated cross-sectional prevalences of BD in individuals aged >10 years in Kayseri, Turkey, in two stages. The first stage aimed to identify individuals with recurrent oral ulcers (ROUs) through home visits, and the second stage aimed to further examine those with ROUs for the presence of other BD-related manifestations under hospital conditions. The study was conducted using the criteria defined by the International Study Group for Behcßet’s Disease. The sample size was determined to be 4697 with an expected sampling error of 5.5 per 10,000, with a 95% confidence interval. A standard questionnaire was administered to a total of 5218 individuals. Results A history of ROU was recorded in 470 (9.0%) of the 5218 residents, and a previous diagnosis of BD was recorded in nine individuals. The prevalence rate of BD was estimated as 17 cases per 10,000 population in Kayseri, Turkey. Conclusions The present study contributes towards estimations of prevalences of BD in Turkey and towards raising public awareness about the disease. It also supports previous studies that have reported the world’s highest prevalences of BD in Turkey.
Introduction
286
Behcßet’s disease (BD) was first described by Hulusi Behcßet in 1937 in three patients with a triple symptom complex of aphthae, genital ulcers, and hypopyon uveitis.1 It is a chronic multisystem disorder characterized by recurring inflammatory processes.2,3 Although BD has been reported all over the world, it has a distinct geographic distribution. Reported rates show regional differences within and between countries. It occurs most frequently between the latitudes of 30 °N and 45 °N in Asian and European populations; geographically this suggests a possible relationship with the historic silk trading route. Large epidemiological surveys are lacking, and prevalence figures in many countries are based on case registries and hospital attendance.4,5 In Turkey, five different surveys6–10 have reported prevalence rates in International Journal of Dermatology 2015, 54, 286–289
the range of 20–421 per 100,000 population, according to which Turkey is ranked as the country with the highest rate of BD in the world. However, these surveys6–10 were carried out in different regions of Turkey and screened selected rural areas or urban areas (Table 1). A nationwide, population-based study establishing the prevalence of BD in Turkey is lacking. The present study was conducted to aid in estimating the prevalence rate of BD in Turkey and to raise public awareness of the disease. Materials and methods Study design
This study investigated cross-sectional prevalences of BD in individuals aged >10 years in Kayseri, a city located in the center of Anatolia. The study was carried out in two ª 2014 The International Society of Dermatology
C ß o€ lgecß en et al.
Prevalence of Behc ß et’s disease in a Turkish city
Report
Table 1 Field surveys of Behc ßet’s disease (BD) conducted in Turkey
Year, authors 1981, 1988, 2002, 2003, 2004,
Demirhindi et al.6 Yurdakul et al.7 Idil et al.8 Azizlerli et al.9 Cakir et al.10
Region, city, all field surveys
BD patients, n
Patients aged >10 years, n
Prevalence per 100,000 population
Istanbul, rural (Northeast), Ordu, rural Ankara, suburban Istanbul, urban (Thrace), Edirne, rural
4 19 16 101 1
4960 5131 13 894 23 986 (>12 years) 4861
80 370 115 421 20
stages. The first stage identified individuals with recurrent oral ulcers (ROUs) through home visits; the second stage further examined those with ROUs for the presence of other BD-related manifestations under hospital conditions. The first stage of the study
The population of Kayseri was reported to be 1,060,432 in the Turkish census carried out in 2000. The sample size was determined to be 4697 with an expected sampling error of 5.5 per 10,000, with a 95% confidence interval (CI). A total of 5218 individuals aged >10 years and living in the city center and the districts of Kayseri were included in this study. The areas covered by 30 primary healthcare centers in the city center and in 14 districts of Kayseri were selected to represent the study site using a simple random sampling method. Individuals to be included in this study were identified using a systematic sampling method from the medical records of the primary healthcare centers selected. The number of individuals to be screened in each primary healthcare center area was determined in proportion to the population covered by all primary healthcare centers in Kayseri. The 5218 individuals were interviewed using a standard questionnaire administered by dermatologists. The standard questionnaire used in this study was prepared mainly for the purposes of identifying individuals with ROUs or those with a diagnosis of BD. All individuals with ROUs and those who had been diagnosed previously with BD elsewhere were invited to attend the BD Outpatient Clinic at the Department of Dermatology, Erciyes University, Kayseri, for further investigation.
In this study, BD was diagnosed according to the criteria defined by the International Study Group for Behcßets Disease (ISGBD)11 (Table 2). Results The study was comprised 5218 residents aged from 10 years to 83 years. The mean standard deviation (SD) age was 33.8 14.7 years. A history of ROUs was recorded in 470 (9.0%) participants, and a previous diagnosis of BD was recorded in nine individuals. In the second stage of the study, 442 of the 470 (94.0%) ROU patients were confirmed as having true ROUs; the remainder were found to have other oral lesions, such as herpes simplex stomatitis, mucosal ulceration caused by dental trauma, scrotal tongue, oral lichen planus, and pemphigus vulgaris. Individuals with true ROUs were examined for other symptoms of BD (genital ulcers, erythema nodosum, acneiform lesions, folliculitis,
Study Group for Behcßet’s Disease diagnostic (classification) criteria11 (1990)a
Table 2 International
Recurrent oral ulceration Plus two of: Recurrent genital ulceration Eye lesions
Second stage of the study
In this stage of the study, individuals who had indicated the presence of ROUs or BD during the first stage of the study were examined for other BD-related manifestations. This investigation included a physical examination and skin pathergy test. All individuals with ROUs and one of the other BD-related manifestations underwent a complete ophthalmological examination by a ophthalmologist at the Uveitis Outpatient Clinic, Department of Ophthalmology. ª 2014 The International Society of Dermatology
Skin lesions
Pathergy
Minor aphthous, major aphthous, or herpetiform ulceration observed by the physician or patient and recurring at least three times in one 12-month period
Aphthous ulceration or scarring, observed by the physician or patient Anterior uveitis, posterior uveitis, cells in the vitreous on slit-lamp examination, or retinal vasculitis observed by the ophthalmologist Erythema nodosum observed by the physician or patient, pseudofolliculitis, papulopustular lesions or acneiform nodules observed by the physician in post-adolescent patients not on corticosteroid treatment Read by the physician at 24–48 h
a
Findings are applicable only in the absence of other clinical explanations. International Journal of Dermatology 2015, 54, 286–289
287
288
Report
C ß o€ lgecß en et al.
Prevalence of Behc ß et’s disease in a Turkish city
arthritis). Of the 442 patients with true ROUs, pathergy tests were performed in 420 (95.0%). Of these 420 patients, results were negative in 328 (78.1%) patients. Ophthalmological examinations were performed in 42 patients with ROUs and one of the other BD-related manifestations. A diagnosis of BD according to the ISGBD criteria11 was established in nine individuals. All nine of these patients had been previously diagnosed with BD and had been followed up at various centers. Of these nine patients, four (44.4%) were female and five (55.6%) were male. Their ages ranged between 27 years and 58 years (mean SD age: 40.9 10.4 years). Clinical findings in the nine patients are summarized in Table 3. Recurrent oral ulcers were observed in all of the nine patients, genital ulcers in eight, ocular involvement in three, arthritis in one, and arthralgia in five. Skin manifestations in these nine patients included pseudofolliculitis in six patients, erythema nodosum in four patients, and extragenital ulcers in one patient. The skin pathergy test was positive in four (44.4%) of the nine patients. The prevalence of mainly mucocutaneous involvement was 44.4%, whereas the prevalence of vital organ involvement was 55.6% (five patients, including three male and two female patients, of whom three had ocular involvement and two had deep venous thrombosis). A family history of BD was recorded in four (44.4%) of the nine patients. In conclusion, the prevalence of BD in Kayseri, Turkey, was calculated as 9/5218 = 0.001725 and thus as 17 cases per 10,000 population (95% CI 8–33) in this survey. Discussion Behcßet’s disease is common in those countries that extend from the Mediterranean to the Far East along the ancient trading route known as the Silk Road. A nationwide Table 3 Clinical features in patients diagnosed with Behc ßets
disease
Recurrent oral ulcers Genital ulcer Eye involvement Pseudofolliculitis Erythema nodosum Arthritis Arthralgia Deep vein thrombosis Extragenital ulcers Positive pathergy test
n (F, M)
%
9 8 3 6 4 1 5 2 1 4
100 88.9 33.3 66.6 44.4 11.1 55.5 22.2 11.1 44.4
(4, (3, (1, (3, (3, (0, (2, (1, (0, (1,
5) 5) 2) 3) 1) 1) 3) 1) 1) 3)
F, female; M, male. International Journal of Dermatology 2015, 54, 286–289
survey based on hospital records revealed an estimated prevalence of BD in the Japanese population of 7–8.5 cases per 100,000 population.1–3 One field survey showed a prevalence of 17 cases per 100,000 population in Iraq, and another reported a frequency of 120 cases per 100,000 population in an Arab community in Israel.12,13 These frequencies are distinctly higher than that found among ethnic Jews.12,13 Estimated prevalences elsewhere range from 0.64 per 100,000 population in the UK and 6.4 per 100,000 population in Spain, to 8.6 per 100,000 population in the USA.3 Five previous studies have focused on the frequency of BD in Turkey; reported prevalences ranged from 20 to 421 per 100,000 population.6–10 Prevalences of BD were found to be 80 per 100,000 in Silivri near Istanbul,6 370 per 100,000 in C ß amasß in northern Anatolia,7 110 per 100,000 in the Park region of Ankara,8 421 per 100,000 in Istanbul,9 and 20 per 100,000 in a rural population in Havsa.10 Apart from the differences in prevalence, differences in the frequencies of various manifestations of BD were also identified in these studies. No individuals with ocular involvement were identified in C ß amasß and Havsa,7,10 but the frequency of ocular involvement was reported to be 50.0% in Silivri,6 43.7% in the Park region of Ankara,8 27.7% in Istanbul,9 and 33.3% in the present study. The frequency of a positive pathergy test was 33.3% in Silivri and C ß amasß,6,7 81.2% in the Park 8 region of Ankara, 69.3% in Istanbul,9 and 44.4% in this study. Findings of a male predominance in patients with a severe course of disease are consistent with previous reports.2,3,9 The varying prevalence rates of BD in Turkey (20–421 per 100,000 population) may relate to regional differences, different sample sizes, and particular urban and rural characteristics. The present study included both rural and urban areas, but three of the areas studied previously (C ß amasß, Silivri, Havsa) were rural, and the other two (Ankara, Istanbul) were suburban or urban. Istanbul is a large cosmopolitan city with very high population motility. This phenomenon might contribute to increases in the prevalence of BD, as well as of other diseases. The populations of C ß amasß, Ankara, and Kayseri largely consist of Anatolian Turks, whereas the population of Havsa is more homogeneous and includes a higher proportion of Balkan Turks. The lower prevalence identified in Havsa may relate to the ethnic distribution of its population. Although ROUs represent the symptom most frequently seen in BD, in the present study only nine of 442 individuals with ROUs (20 per 1000 population) were found to have BD. Findings in the other studies reported from Turkey varied. In C ß amasß, 19 of 417 individuals with ROUs (45 per 1000 population) had BD.7 In Ankara, 16 ª 2014 The International Society of Dermatology
C ß o€ lgecß en et al.
of 823 individuals with ROUs (19 per 1000 population) had BD.8 In Istanbul, 101 of 700 individuals with ROUs (144 per 1000 population) had BD,9 and in Havsa, one of 115 individuals with ROUs (9 per 1000 population) had BD.10 In a study from South Korea, Bang and colleagues observed that 50% of patients with ROUs developed BD.14 However, unlike the Turkish studies,6–10 which were based on field surveys, the study reported by Bang et al.14 was based on hospital records. Thus disparities between the results of studies from the two countries may be attributable to dissimilarities in their methods, and to ethnic, genetic, and geographical differences. In conclusion, the prevalence of BD varies among different countries. In Turkey, a nationwide, populationbased study investigating the prevalence of BD is lacking. The fact that this study found no new cases of BD may be explained by the increased healthcare facilities available in Kayseri. The present study will contribute towards the estimation of prevalence rates of BD in Turkey and will help to raise public awareness of this disease. Its findings also support those of previous studies that have reported Turkey as having the world’s highest prevalence of BD6–10. References 1 Behcßet H. Uber rezidivierende, aphthose, durch ein Virus verursachte Geshwure am Munde, am Auge und an den Genitalien. Dermatol Wochenschr 1937; 36: 1152–1157. 2 Sakane T, Takeno M, Suzuki N, et al. Behcßets disease. N Engl J Med 1999; 41: 1284–1291. 3 Yurdakul S, Yazici H. Behcßets syndrome. Best Pract Res Clin Rheumatol 2008; 22: 793–809.
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4 James DG. Silk route disease. Postgrad Med J 1986; 62: 151–153. 5 Verity DH, Marr JE, Ohno S, et al. Behcßets disease, the Silk Road and HLA-B51: historical and geographical perspectives. Tissue Antigens 1999; 54: 213–220. 6 Demirhindi O, Yazıcı H, Binyıldız P, et al. Silivri Fener Koyu ve yoresinde Behcßet Hastalı gı sıklı gı ve bu hastalı gın toplum icinde taranmasında kullanılabilecek bir yontem. Cerrahpasa Tıp Fak Derg 1981; 12: 509–514. 7 Yurdakul S, Gunaydin I, Tuzun Y, et al. The prevalence of Behcßets syndrome in a rural area in northern Turkey. J Rheumatol 1988; 15: 820–822. 8 Idil A, Gurler A, Boyvat A, et al. The prevalence of Behcßets disease above the age of 10 years. The results of a pilot study conducted at the Park Primary Health Care Center in Ankara, Turkey. Ophthalmic Epidemiol 2002; 9: 325–331. 9 Azizlerli G, Kose AA, Sarica R, et al. Prevalence of Behcßets disease in Istanbul, Turkey. Int J Dermatol 2003; 42: 803–806. 10 Cakir N, Dervis E, Benian O, et al. Prevalence of Behcßets disease in rural western Turkey: a preliminary report. Clin Exp Rheumatol 2004; 22: 53–55. 11 International Study Group for Behcßets Disease. Criteria for diagnosis of Behcßets disease. Lancet 1990; 335: 1078–1080. 12 Al-Rawi ZS, Neda AH. Prevalence of Behcßets disease among Iraqis. Adv Exp Med Biol 2003; 528: 37–41. 13 Jaber L, Milo G, Halpern GJ, et al. Prevalence of Behcßets disease in an Arab community in Israel. Ann Rheum Dis 2002; 61: 365–366. 14 Bang D, Yoon KH, Chung HG, et al. Epidemiological and clinical features of Behcßets disease in Korea. Yonsei Med J 1997; 38: 428–436.
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