베체트 증후군의 역학적 고찰

Abstract

[한글]

베체트 증후군은 1937년 Hulusi Behcet이 처음 기술한 질환으로 구강 및 외음부궤양, 다양한 피부병변과 안증상등을 주증상으로 하며 원인불명의 반복적인 염증반응을 특징으로 한다.

본 질환에 대한 원인, 발생기전 및 유병율은 아직 확실히 알려져 있지 않다.

저자는 베체트 증후군에 대한 역하걱 연구를 위해 세브란스병원 베체트 증후군 특수진료실에 내원한 환자를 Shimizu분류법에 따라서 분류하였다. 그중에서 가능형을 제외한 완전형, 불완전형 및 용의형환자 572명을 대상으로 개인적 특성, 지리적 특성, 사회경제적

특성, 가족력, 임상적특성에 대하여 분석하여 다음과 같은 결과를 얻었다.

1. 분류아형은 불완전형(46.3%)이 가장 많았고, 용의형(36.7%), 완전형(17.0%) 순이었고, 남여비는 0.6:1이었다.

2. 평균 발생연령은 남자 29.1세, 여자 27.1세였다.

3. 년도별 발생은 1955년부터 1970년까진는 10명 미만이엇으나 그후 증가하는 경향을 보였다.

4. 지리적 분포는 서울이 가장 많았고, 증상발현시 거주자와 현거주지 사이의 지리적 분포는 차이가 없었고, 주로 도시에 거주하였다.

5. 직업별 분포는 남자는 사무직(31.5%), 운번(10.6%), 노무직(7.9%), 교사(7.4%), 기술직(6.5%)순이었고, 여자는 사무직(6.7%), 교사(3.7%), 노무직(2.8%), 기술직(2.5%), 무

직(81.8%) 이었다.

6. 구강내 자극인자로 생각되는 요인으로는 재발성 편도선염(20.8%)이 가장 많았고, 계절별로는 여름에 악화되는 환자가 (8.4%) 가장 많았다.

7. 월경시에 악화되는 경우는 22.2%의 환자에서 나타났는데 시기는 월경전(12.8%), 월경중(5.3%), 월경후(4.8%)순이었다.

8. 최초발생 주증상은 구강궤양(80.6%)이 가장 많았고, 피부증상(10.3%), 외음부궤양(6.3%), 안증상(2.8%)순이었다.

9. 최촬생 주증상과 그다음 발생주증상 발현시 까지의 평균기간은 완전형이 5.2년, 불완전형이 6.3년, 용의형이 6.3년이었다.

10. 내원당시 주증상의 평균유병기간은 구강궤양 6.4년, 외음부궤양 3.2년, 피부증산 3.1년, 안증상 2.6년으로 구강궤양의 유병기간이 가장 길었다.

11. 매년 주증상의 평균 재발회수는 구강궤양 14.6회/년, 외으부궤양 7.2회/년, 피부증상 8.0회/년, 안증상 4.8회/년으로 구강궤양의 재발이 가장 빈번햇다.

12. 한 환자에서 가지고 있던 주증상의 조합은, 불완전형은 구강궤양, 외음부궤양, 피부증상의 조합(65.3%)이 가장 많았고, 용의형은 구강궤양, 피부증상의 조합(54.8%)이 가

장 많았다.

13. 가족중 재발하는 구강궤양이나 베체트 증후군을 갖고있는 빈도는 어머니(10.4%), 형제 및 자매(7.7%), 아버지(2.6%), 배우자(1.4%)순이었다.

이상의 결과로 보아 베체트증후군은 20대후반의 여자에서 많이 발생하며 거주지는 대도시가 많았다.최초발생 주증상은 구강궤양이 가장 많았고, 구강궤양의 재발이 가장 빈번했으며, 구강궤양의 평균 유병기간이 가장 길엇고 최초 주증상 발생후 제 2의 주증상 발생

은 5~6년 걸렸다. 외국의 보고서와의 차이는 우리연구에서 여자의 비율이 높은 점이다.

Epidemiologic findings of Behcet's Syndrome

Moo Yon Cho

Department of Medical Science, The Graduate School, Yonsei University

(Directed by ProfessorSungnack Lee, M.D.)

Behcet's syndrome was first described by Hulusi Behcet in 1937 with the clinical

presentation of frequently recurring oral and enital ulcers, and various skin and

eye lesions of unknown etiology. The etiology, pathogenesis, heredity and

prevalence of this disease are still unknown. For the purpose of epidemiologic

study, the patients who visited Severance Hospital Behcet's Syndrome Speciality

Clinic were grouped by Shimizu classification into the complete, incomplete and

suspected types and evaluated. The results of the evaluation with respect to

personal characteristics, geogrophic distribution, socioeconomic characteristics,

family history and clinical characteristics are as follows.

1. The incidence among the subgroups was incomplete type (46.3%), suspected type

(36.7%), complete type (17.0%) and the male to female ratio was 0.61:1.

2. The average age of onset was 29.1 years for males and 27.6 years for females.

3. The yearly incidence was less than 10 cases per year until 1970. When it began

to increase, reaching a maximum of 78 cases in 1983.

4. Regionally Seoul had the greatest number of patients, and there was no

significant difference first occurred and the present address.

5. The occupational distribution among males included office workers, 31.5%;

drivers, 10.6%; laborors, 7.9%; teachers, 7.4$; engineers, 6.5%, and among females

was office workers, 6.7%; labrors, 2.8%; unemployed, 81.7%.

6. Among the presumed aggrevation factors, oral ulcers and recurent tonsillitis

(20.8%) were the most frequent and aggrevation of the disease occurred more

frequently in the summer (8.4%).

7. Excerbation in realation to menstruation was premenstrual (12.8%), during

menstruation (5.3%) and postmenstrual (4.8%).

8. The initial symptoms were oral ulcers (80.6%), skin lesions(10.3%), genital

ulcer (6.3%) and eye symptoms (2.8%).

9. The average duration between the appearance of the first and second major

manifestations was 5.2 years for the complete type, 6.3 years for the incomplete

type and 6.3 years for the suspected type, 6.3 years for the incomplete type and

6.3 years for the suspected type.

10. The average duration of the symptoms at the time of diagnosis was 6.4 years

for oral ulcer, 3.2 years for genital ulcers, 3.1 years for skin lesions and 2.6

years for eye lesions, with the oral ulcers showing the longest duration of

symtoms.

11. The rate of symptom recurrence per years were for genital ulcers, 8.0

times/years for skin lesion and 4.8 times/years for eye lesions.

12. Combinations of major manifestations most commonly included oral ulcers,

genital ulcers and skin lesions (65.3%) for the incomplete type, and oral ulcers

and skin lesions (54.8%) for the suspected type.

13. The existence of recurrent oral ulcers of Behcet's syndrome in the families

of patients with Behcet;s syndrom was 10.0% for mothers, 7.7% for siblings, 5.2%

for fathers and 1.4% for partners.

[영문]

Behcet's syndrome was first described by Hulusi Behcet in 1937 with the clinical presentation of frequently recurring oral and enital ulcers, and various skin and eye lesions of unknown etiology. The etiology, pathogenesis, heredity and prevalence of this disease are still unknown. For the purpose of epidemiologic

study, the patients who visited Severance Hospital Behcet's Syndrome Speciality Clinic were grouped by Shimizu classification into the complete, incomplete and suspected types and evaluated. The results of the evaluation with respect to personal characteristics, geogrophic distribution, socioeconomic characteristics, family history and clinical characteristics are as follows.

1. The incidence among the subgroups was incomplete type (46.3%), suspected type (36.7%), complete type (17.0%) and the male to female ratio was 0.61:1.

2. The average age of onset was 29.1 years for males and 27.6 years for females.

3. The yearly incidence was less than 10 cases per year until 1970. When it began to increase, reaching a maximum of 78 cases in 1983.

4. Regionally Seoul had the greatest number of patients, and there was no significant difference first occurred and the present address.

5. The occupational distribution among males included office workers, 31.5%; drivers, 10.6%; laborors, 7.9%; teachers, 7.4$; engineers, 6.5%, and among females was office workers, 6.7%; labrors, 2.8%; unemployed, 81.7%.

6. Among the presumed aggrevation factors, oral ulcers and recurent tonsillitis (20.8%) were the most frequent and aggrevation of the disease occurred more frequently in the summer (8.4%).

7. Excerbation in realation to menstruation was premenstrual (12.8%), during menstruation (5.3%) and postmenstrual (4.8%).

8. The initial symptoms were oral ulcers (80.6%), skin lesions(10.3%), genitalulcer (6.3%) and eye symptoms (2.8%).

9. The average duration between the appearance of the first and second major manifestations was 5.2 years for the complete type, 6.3 years for the incomplete type and 6.3 years for the suspected type, 6.3 years for the incomplete type and 6.3 years for the suspected type.

10. The average duration of the symptoms at the time of diagnosis was 6.4 years for oral ulcer, 3.2 years for genital ulcers, 3.1 years for skin lesions and 2.6 years for eye lesions, with the oral ulcers showing the longest duration of symtoms.

11. The rate of symptom recurrence per years were for genital ulcers, 8.0 times/years for skin lesion and 4.8 times/years for eye lesions.

12. Combinations of major manifestations most commonly included oral ulcers, genital ulcers and skin lesions (65.3%) for the incomplete type, and oral ulcers and skin lesions (54.8%) for the suspected type.

13. The existence of recurrent oral ulcers of Behcet's syndrome in the families of patients with Behcet;s syndrom was 10.0% for mothers, 7.7% for siblings, 5.2% for fathers and 1.4% for partners.