ABSTRACT
Behçet’s disease (BD) is a disease that is characterized by recurrent oral aphthae and has various involvements especially in eye, skin and vascular structures. It was presented to the modern medical community by Hulusi Behçet in 1937. Although the prevalence of BD varies according to the population, it is mostly diagnosed in Türkiye. BD in patients with recurrent oral aphthae (at least three times in one year) plus two of the following clinical features: recurrent genital aphthae, eye lesions, skin lesions and a positive pathergy test. In the literature, data on BD diagnosed during pregnancy could not be reached. Our case was referred to the dentist at the 17th week of her pregnancy because of the wounds in her mouth. Then, after evaluation with a multidisciplinary approach, BD was diagnosed and treatment was started. In the literature, the effect of pregnancy on the disease or the effect of the disease on pregnancy has been investigated. Our case is the first in the literature to be diagnosed during pregnancy, a period when the disease is generally in remission.
Introduction
Behçet’s disease (BD) is a disease that is characterized by recurrent oral aphthae and can have various involvements especially in the eye, skin and vascular structures.It was reported to be first described by Hippocrates, but was introduced to the modern medical community by Hulusi Behçet in 1937 (1, 2).
The prevalence of BD varies according to the population (3). The prevalence of the disease, which is mostly diagnosed in Türkiye, varies between 80 and 370 cases per 100,000 (3).
The diagnostic criteria of BD are as follows; patients with recurrent oral aphthae (at least three times in one year) plus two of the following clinical features: recurrent genital aphthae, eye lesions, skin lesions and a positive pathergy test (4).
Although the etiology is not known exactly, it is histopathologically characterized by vasculitis and vasculitis is seen in all affected organs. In addition, pathophysiologies associated with autoimmunity, microbiological agents and HLA-B51 have been suggested (5, 6).
Data on BD diagnosed during pregnancy could not be reached in the literature. In general, the effect of pregnancy on the disease or the effect of the disease on pregnancy was investigated and the results were given.
However, we wanted to present this case in order to show that such an important diagnosis can be made during pregnancy with a detailed medical history and to show that we can possibly prevent any complications during pregnancy with treatment.
Case Report
A 26-year-old G3P2A0 patient was admitted to our department at her 17th gestational week with complaints of intraoral sores.
The patient, whose medical and surgical history was unremarkable, did not have any obstetric complaints. Only, it was learned that the other two pregnancies occurred as preterm delivery at 36 weeks of gestation.
In the obstetric evaluation, a fetus compatible with the gestational week was observed in the intrauterine regular gestational sac, and no major structural anomaly was observed in the fetus.
The patient was first evaluated in detail by the periodontologist with her current complaints, and in the medical history taken, it was learned that the ulcerated lesions in the mouth recurred 4-5 times during the year. When the anamnesis was deepened, it was learned that the patient had new-onset joint swelling, as well as low back pain that started in the pre-pregnancy period but continued to increase in severity. It was learned that the patient also had pustular lesions similar to the lesions in the mouth from time to time in the genital area. In previous dermatology examinations, it was documented that papulopustular and acneiform lesions persisted on her skin.
Upon the suggestion of the periodontologist, BD was suspected and the patient was consulted to the immunology department, and as a result of the examinations and exclusion of other rheumatological diseases, especially systemic lupus erythematosus and rheumatoid arthritis, BD was diagnosed - based on other clinical findings even though pathergy test is negative - and the patient was started on colchicine 2*0.5 mg orally. At the 2-week follow-up evaluation after treatment, the symptoms of low back pain and swelling in the joints improved dramatically.
The pregnancy of the patient, who is at 28 weeks of gestation, continues with colchicine treatment and without any symptoms and problems. Informed consent was obtained.
Discussion
In the literature, many studies have shown that BD goes into remission during pregnancy, but it has been reported that the risk of complications is higher than those who do not have the disease (7-10).
In the study of Noel et al., (8) exacerbation was observed in 36% of pregnancies, and the annual incidence of exacerbations per patient was shown to be lower than before and after pregnancy. In the same study, the overall complication rate was reported as 16%, among which miscarriages, HELLP syndrome, and preterm birth were reported. Complications were reported to be significantly less common in patients receiving colchicine.
A case-control study of 31 patients during 135 pregnancies demonstrated higher rates of miscarriage, pregnancy complications, and cesarean sections in patients with BD compared with controls (7).
In a retrospective analysis conducted in Türkiye in 2018, it was shown that there was a history of 24.2% miscarriage, 3% intrauterine death, and 24% preterm birth (10).
Preterm delivery occurred in the previous 2 pregnancies of our case, and both babies of the patient were delivered at 36 weeks of gestation.
Due to the existence of cases of BD associated with preterm birth in the literature, the following question comes to mind. Perhaps the patient also had this disease in her previous pregnancies, and if she had been diagnosed at that time and treatment had been started, the delivery would not have occurred at 36 weeks of gestation.
Therefore, it is very important to take a medical history and work multidisciplinary. We understand this even more in this case.
BD is an immunological disease that can be recognized and treated with a proper medical history, even during pregnancy, when it is generally in remission, and the complication rate is very low when treatment is started.
