A case of pediatric Behçet's disease with intestinal involvement

We report a 6-year-old girl with recurrent oral aphthae, genital and perianal ulcers, and folliculitis-like erythema followed by intestinal symptoms. No ocular lesions were found. Her ailment was diagnosed as incomplete Behçet's disease with intestinal involvement. According to the literature, pediatric Behçet's disease is characterizesd by a low incidence of ocular lesions and a high incidence of intestinal involvement, as exhibited in this case. As intestinal lesions in pediatric Behçet's disease are often life-threatening, a barium enema must be given to exclude the possibility of intestinal involvement when pediatric patients with Behçet's disease complain of abdominal pain     

Evaluation of clinical findings according to sex in 2313 Turkish patients with Behçet's disease

Background Behçet's disease is a multisystem disease featuring mucocutaneous, ocular, articular, vascular, intestinal, urogenital, and neurologic involvement and occurs with a high prevalence in the Mediterranean including Turkey. Higher incidence of severe clinical course and systemic involvement is observed in male patients. Objective To determine the influence of sex on the clinical course of Behçet’ s disease. Methods We retrospectively evaluated the clinical findings of 2313 Behçet patients followed up at the multidisciplinary Behçet's Disease Center at Ankara University. Results The male/female patient ratio was 1.03. Oral aphthae was seen in all patients. In male Behçet patients, the prevalence of mucocutaneous lesions and systemic manifestations was as follows: 85.6% genital aphthae, 45.5% erythema nodosum, 59.5% papulopustular lesions, 17.5% thrombophlebitis, 38.1% ocular involvement, 11.3% articular involvement, 11.7% vascular involvement, 3.3% neurologic involvement, 1.4% gastrointestinal involvement, and 1.8% pulmonary involvement. In female Behçet patients, the prevalence of manifestations were as follows: 91% genital aphthae, 49.8% erythema nodosum, 48.3% papulopustular lesions, 3.5% thrombophlebitis, 19.8% ocular involvement, 11.8% articular involvement, 2.1% vascular involvement, 1.3% neurologic involvement, 1.4% gastrointestinal involvement, and 0.03% pulmonary involvement. Conclusions Only genital aphthae and erythema nodosum were more frequent in females. On the other hand papulopustular eruptions, thrombophlebitis, ocular, neurologic, pulmonary and vascular involvement were more frequent in males. While female patients had the best prognosis, male patients had a worse overall prognosis than females.     

Guidelines for the treatment of skin and mucosal lesions in Behçet's disease: A secondary publication

In the current study, we present guidelines for the diagnosis and treatment of the mucocutaneous lesions of Behçet's disease, which is a chronic inflammatory disease characterized by the involvement of various organs, including mucocutaneous, ocular, vascular, intestinal and central nervous system lesions. It is often identified in the Middle East Mediterranean to East Asia region. Skin manifestations include erythema nodosum, papulopustular lesions and thrombophlebitis, and mucosal manifestations include oral and genital ulcers. These mucocutaneous lesions are characteristically the first signs of Behçet's disease and are important to be recognized for the early diagnosis of the disease. Moreover, these manifestations also recur and persist over the long-term course of the disease. The management of mucocutaneous lesions is important to prevent recurrence. We developed consensus guidelines that provide recommendations for general practitioners and dermatologists and physicians on the management of the mucocutaneous lesions of Behçet's disease.     

Colchicine in the treatment of the cutaneous manifestations of Behçet's disease

Five patients with Behçet's disease were satisfactorily treated with colchicine. Oral aphthosis, erythema nodosum-like lesions and genital erosions improved greatly within a month as did laboratory findings. We believe colchicine to be the first choice in the management of the cutaneous and ocular lesions of Behçet's disease.     

The clinical course of Behçet's disease in pregnancy: a retrospective analysis and review of the literature

Although Behçet's disease is mainly diagnosed during the reproductive periods of life, we know little about the influence of pregnancy on the clinical course of Behçet's disease. Therefore, we analyzed the relationship between Behçet's disease and pregnancy retrospectively, in order to detect any possible interaction between the two multisystemic processes, particularly in regard to the influence of pregnancy on the clinical course of Behçet's disease. We studied 44 pregnancies in 28 women with Behçet's disease. The diagnoses were made according to the criteria of the International Study Group for Behçet's disease. The patients were observed during pregnancy and puerperium periods at monthly intervals. The existence and incidence of symptoms were recorded during these periods. There was remission of Behçet's disease during 23 (52.3%) pregnancies, although the disease had been in a stage of exacerbation before pregnancy. The disease became exacerbated during 12 (27.3%) pregnancies, although it had been in a stage of remission before pregnancy. There were no changes in the clinical course of Behçet's disease in 9 (20.4%) pregnancies. The most frequent manifestations of the clinical exacerbation were increases in the intensity and severity of outbreaks of oral ulcers during pregnancy. Outbreaks of genital ulcers, eye inflammations, and arthritis were other signs of exacerbation. Other than spontaneous abortion in three patients, we did not observe maternal or fetal complications. Although Behçet's disease tends toward remission during pregnancy, the influence of pregnancy on its clinical course is quite variable between patients and even during different pregnancies in the same patient. On the basis of our and previous results, we speculate that pregnancy in general does not seem to markedly affect the natural course of Behçet's disease.     

Association of class I HLA antigens with the clinical manifestations of Turkish patients with Behçet's disease

Summary Genetic factors appear to be important in the pathogenesis of Behçet's disease. Although it is known to be strongly associated with HLA‐B51, the association of HLA class I antigens with specific clinical findings of the disease has not been studied extensively and the few studies are conflicting. The aim of this study was to investigate the association of HLA class I alleles with the manifestations of Behçet's disease in Turkish patients. Eighty‐five patients with Behçet's disease were typed for HLA‐A, B, and C antigens with the serologic, standard microlymphocytotoxicity technique. Possible associations of the HLA complex with clinical findings of Behçet's disease were examined. Statistically significant findings are as follows (P < 0.05): increased HLA‐B51 and decreased HLA‐B35 frequency in patients with thrombophlebitis, increased HLA‐A29 and decreased HLA‐Bw6 frequency in patients with ocular involvement, decreased HLA‐Cw2 frequency in patients with erythema nodosum, and decreased HLA‐Cw7 frequency in patients with genital ulceration. Of particular note, the results of this study suggest that the presence of HLA‐B51 and the absence of HLA‐B35 can be regarded as laboratory risk factors of venous thrombosis in patients with Behçet's disease.     

The frequency of scarring after genital ulcers in Behçet's syndrome: a prospective study

Background Although genital ulceration with or without scar formation is an important manifestation of Behçet's syndrome, formal data on the rate of genital scarring are lacking. Objective To study prospectively the frequency of genital scar formation after genital ulcers in patients with Behçet's syndrome. Methods One hundred and two patients (72 males and 30 females) with fresh genital ulcers presenting at a dedicated Behçet's syndrome outpatient clinic were followed for 6 weeks. The ulcer location, size, and frequency of scar formation were noted. All patients were treated with similar topical therapy. Results Two hundred and ten genital ulcers were followed in both sexes. The rate of scar formation was 66.2% (102/154) in male patients and 60.7% (34/56) in female patients. When genital ulcers were stratified as a function of size, large genital ulcers (≥ 1 cm in diameter) developed scars more frequently than did smaller ulcers (< 1 cm). The rate of scar formation in male patients with large genital ulcers was 89.4% (59/66) and with small ulcers was 48.9% (43/88) (P < 0.001). In females, all 19 large ulcers healed with scarring, and the same was true for 40.5% (15/37) of the small ulcers (χ² = 18.61, P < 0.001). Conclusion About two‐thirds of genital ulcers in Behçet's syndrome leave scars. The rate of scar formation depends on the ulcer size.     

Histopathologic Study of Cutaneous Lesions in Behçet's Syndrome

Light microscopic studies of 55 patients with complete and incomplete types of Behçet's syndrome were reviewed. We reported here 39 skin biopsies from 30 patients with cutaneous lesions of oral, and genital ulcers, folliculitis, erythema multiforme, and thrombophlebitis. Leukocytoclastic vasculitis was seen in 5 of 12 (42%) cases of oral ulcers and 2 of 7 (29%) of genital ones. Lymphocytic vasculitis was demonstrated in 3 of 12 (25%) cases of oral ulcers and 4 of 7 (57%) of genital ulcers. However, the vasculitis was observed only locally in the areas with moderate or severe inflammation. From the biopsy specimens of our cases with fully developed mucocutaneous lesions of Behçet's syndrome, it appears that the forms of vasculitis that are often emphasized in the literature are a phenomenon secondary to the intense inflammation seen in such cutaneous lesions.     

Expression of bcl-2 protein in active skin lesions of Behçet's disease

Abstract Background Expression of bcl‐2 protein has been shown to play an important role in the pathogenesis of some inflammatory as well as neoplastic disorders. In this study we have investigated the presence of bcl‐2 protein in active skin lesions of Behçet's disease and compared these results with normal skin samples of Behçet's disease (BD) patients and BD unrelated leukocytoclastic vasculitis. Methods Active skin lesions of 23 Behçet's disease patients, normal skin samples of seven Behçet's disease patients, and archival biopsy specimens of 23 cutaneous leukocytoclastic vasculitis were investigated for the presence of bcl‐2 protein by immunohistochemical methods. Results of staining were assessed semiquantitatively. Chi‐square tests were used for statistical analysis. Results Expression of bcl‐2 protein were demonstrated in 16 of 23 (69.5%) and 8 of 23 (34.7%) patients with Behçet's disease and leukocytoclastic vasculitis, respectively. There were statistically significant difference between two groups (x² = 4.27, P < 0.05). None of the normal skin samples of Behçet's disease patients showed bcl‐2 expression. Conclusion Expression of bcl‐2 protein may play a particular role in the development of skin lesions in Behçet's disease by causing prolonged survival of infiltrating lymphocytes.     

JUVENILE BEHÇET'S DISEASE AMONG 1784 TURKISH BEHÇET'S PATIENTS

Background. Behçet's disease is a chronic, relapsing disease, about which information on its clinical course in juveniles is only available from small groups of patients. Materials and Methods. Patients suffering from Behçet's disease who had their first lesion at or before the age of 16 were evaluated in terms of: age at onset, mucocutaneous signs, and findings related to systemic involvement. Ninety-five patients, evaluated as having juvenile Behçet's disease (JBD), were detected among 1784 Turkish Behçet's patients. The mean age of these 95 patients (51 boys or men, 44 girls or women) who had JBD was 26.8 ± 7.1 years. Results. The difference between sexes in terms of age at onset, development period of second lesions, and systemic involvement was not found to be significant in JBD. Patients were divided into two groups, one showing severe disease (N = 27) and the other mild disease (N = 68). There was no significant difference between the two groups with respect to age, age at onset, and sex distribution. The interval between the development of the first and second lesions was shorter in the patient group with severe disease (P < 0.001) and the development of second lesion was most frequently seen in the first 5 years (P < 0.05). Systemic involvement developed also in a shorter time in the group with the severe disease (P < 0.01) and was most frequently encountered during the first 5 years (P < 0.05). Conversely, patients with the mild disease developed systemic involvement more frequently after 6 years or later. Conclusions. Severe Behçet's disease in children and juveniles shows no age or sex predilection, but leads to an earlier recurrence and more severe systemic signs than the mild form.     

Use of Colchicine in the Treatment of Behçet's Disease

ABSTRACT: To date, various treatment modalities have failed to produce consistent improvement in patients having Behçet's disease. Recent reports in the literature, however, have shown promising results with the use of colchicine. Our patient, an 18-year-old white woman, satisfied the 1977 Istanbul Conference criteria for Behçet's disease by exhibiting ocular inflammation and a five-year history of severe, recurrent oral and vaginal ulcerations. She was treated with 0.6 mg of colchicine orally twice daily, and at the end of two weeks of treatment, the oral lesions had completely resolved, and the vaginal ulcers were reduced in diameter and depth to at least one half. Within one month, all oral and Vaginal ulcers were completely healed.     

Fas (CD95) and bcl-2 expression in active skin lesions of Behçet''s disease

Over‐expression of bcl‐2 in lymphocytes has an important role in some immunological and inflammatory diseases. Fas (CD95) is a cell surface molecule that mediates receptor‐triggered apoptosis in various cells including autoreactive T cells. In this study we investigated bcl‐2 and Fas (CD95) expression in dermal lymphocytes in active skin lesions of Behçet's disease (BD) and in skin biopsy samples with chronic, non‐specific inflammations. Tissue sections of 29 skin lesions of Behçet's disease and of 10 chronic non‐spesific inflammatory process cases from the archives of the Ondokuz May?s University's Pathology Department were immunohistochemically stained for bcl‐2 and Fas (CD95), and lymphocytes in the dermal infiltrate were evaluated for cytoplasmic staining. bcl‐2 staining was observed in the skin lesions of 22 cases (75.8%) of Behçet's disease. bcl‐2 staining was detected in two (20%) control skin biopsy samples with non‐specific chronic inflammation. Fas (CD95) positivity was not detected in lymphocytes in Behçet's disease‐related skin lesions. Fas (CD95) staining was observed in only three skin biopsy samples with non‐specific chronic inflammation. bcl‐2 and Fas (CD95) staining values in Behçet's and non‐specific inflammation groups were significantly different (P < 0.01); differences in the bcl‐2 staining values between Behçet's patients with mucocutaneous involvement only and mucocutaneous and other systemic involvements were not significant (P > 0.05). Expression of bcl‐2 and loss of Fas (CD95) expression in dermal lymphocytes may play a role in the development of skin lesions and may account for the chronic course with periodic exacerbations in BD.     

EXPERIMENTAL BEHÇET'S DISEASE AND ULTRASTRUCTURAL X-RAY MICROANALYSIS OF PATHOLOGICAL TISSUES

Environmental chemicals (benzene hexachloride(BHC) and dichlor diphenyl trichlor ethane(DDT) as organic chlorides, Sumithion as an organic phosphorated compound and copper powder) were mixed together and administered orally to pedigreed Pitman-Moor's strain miniature swine for a period of about one year. Oral aphthae, folliculitis, cutaneous and subcutaneous nodules, genital ulcers, hyperemia of conjunctiva and intestinal ulcers were observed in all treated animals. Biopsy and autopsy specimens obtained from the experimental animals showed typical histopathological findings of human Behçet's disease. By ultrastructural X-ray microanalysis of peripheral neutrophils, vascular endothelial cells and infiltrated cells in affected lesions obtained from the experimental animals, highly significant amounts of chlorine and phosphorus were detected, mainly in the lysosomes. The incidence of Behçet's disease has been reported in countries where pesticides made from organic chlorine, organic phosphorus, arsenic and copper compounds have been used in massive amounts. Therefore, our findings suggest that these environmental chemicals play a main role in the pathogenesis of Behçet's disease.     

Clinical features and natural course of Behçet's disease in 661 cases: a multicentre study

Background Behçet’s disease (BD) is a systemic inflammatory disease with unpredictable exacerbations and remissions. The natural course of BD is not fully known. Objectives We aimed retrospectively to determine the occurrence of the symptoms in chronological order. We also evaluated the influence of the treatment and follow‐up on the clinical severity and tried to identify the factors determining severe organ involvement. Methods Six hundred and sixty‐one patients were involved in this multicentre study. The symptoms of the disease were recorded retrospectively in the time order of the manifestations in each patient. Results Oral ulcers were the most common manifestation (100%), followed by genital ulcers (85·3%), papulopustular lesions (55·4%), erythema nodosum (44·2%), skin pathergy reaction (37·8%), and articular (33·4%) and ocular involvement (29·2%). Oral ulcers were the most common onset manifestation (88·7%). The mean ± SD duration between the onset symptom and the fulfilment of diagnostic criteria was calculated to be 4·3 ± 5·7 years. The clinical severity score was significantly increased in the noncompliant treatment group compared with the compliant group with the passage of time (P < 0·001). The frequency of ocular involvement and genital ulcers was significantly higher in patients whose disease onset was at < 40 years. Genital ulcers, ocular involvement, papulopustular lesions, thrombophlebitis and skin pathergy reaction were found to be significantly more frequent in males. Conclusions Mucocutaneous lesions are the hallmarks of the disease, and especially oral ulcers precede other manifestations. The increase in clinical severity score is more pronounced in patients without regular treatment and follow‐up. Male sex and a younger age at onset are associated with more severe disease.     

Can specific vessel‐based papulopustular lesions of Behçet's disease be differentiated from nonspecific follicular‐based lesions clinically?

Background Although papulopustular lesions are one of the diagnostic criteria for Behçet's disease, controversy exists as to the nature of these lesions. Specific vessel‐based papulopustular lesions as well as nonspecific follicular lesions may be seen in patients with Behçet's disease. Some authors suggest that papulopustular lesions should be considered a positive criterion only if they exhibit a vessel‐based neutrophilic reaction. Objective To determine whether specific vessel‐based papulopustular lesions can be differentiated clinically from nonspecific follicular lesions in patients with Behçet's disease. Methods Twenty‐three papulopustular lesions in 20 patients with Behçet's disease were initially examined clinically by two dermatologists blind to each other's diagnosis. Biopsies taken from these lesions were examined by a pathologist unaware of the patient data. Results Leukocytoclastic vasculitis or perivascular infiltration was observed in 10 lesions, perifollicular and perivascular infiltration was noted in nine lesions, and perifollicular inflammation was seen in four biopsy specimens. Most of the lesions interpreted clinically as specific papulopustular lesions of Behçet's disease had predominantly perivascular infiltration or leukocytoclastic vasculitis; however, three papulopustular lesions evaluated by both observers as specific papulopustular lesions had only perifollicular inflammation, and one lesion diagnosed clinically as a nonspecific follicular eruption revealed perivascular neutrophilic reaction. Interobserver variance was noted in three papulopustular lesions. Conclusions Clinical examination may not be sufficient to predict the dermatopathologic pattern in all lesions. Papulopustular lesions with no specific clinical and histopathologic features may create problems in the diagnosis of Behçet's disease.     

Vascular endothelial growth factor levels are increased and associated with disease activity in patients with Behçet's syndrome

Background/aims Vascular endothelial growth factor (VEGF) is a cytokine participating in inflammation with potent endothelial cell effects. It is produced by macrophages, neutrophils and vascular endothelial cells and can alter vessel permeability. Behçet's syndrome is a systemic inflammatory disorder with unknown etiology. Vascular endothelial dysfunction is one of the prominent features of the disease. We previously demonstrated the possible involvement of proinflammatory cytokines [tumor necrosis factor (TNF)‐α, soluble interleukin‐2 receptor (sIL‐2R), interleukin (IL)‐6 and IL‐8], nitric oxide (NO) and adrenomedullin in the etiopathogenesis of Behçet's syndrome. Since VEGF expression is induced by these cytokines and VEGF itself is a potent stimulator of NO production with endothelial cell effects, this study aimed to investigate whether VEGF was affected during the course of Behçet's syndrome. We also assessed the possible involvement of VEGF in ocular Behçet's syndrome or in disease activity. Methods This multicenter case–control study included a total of 39 patients with active (n = 22) or inactive (n = 17) Behçet's syndrome (mean age, 38.1 ± 10.4 years; 21 men and 18 women) satisfying International Study Group criteria, and 15 healthy hospital‐based control volunteers (mean age, 39.2 ± 9.3 years; eight men and seven women) matched for age and gender from a similar ethnic background. Patients were examined by a dermatologist and an ophthalmologist with an interest in Behçet's syndrome. Plasma VEGF concentrations were measured using a newly established enzyme‐linked immunosorbent assay. Clinical findings and acute‐phase reactant parameters such as erythrocyte sedimentation rate, α₁‐antitrypsin, α₂‐macroglobulin, and neutrophil count were used to classify the disease in Behçet's patients as active or inactive. The Wilcoxon test or the Mann–Whitney U‐test was used for statistical analysis as indicated and the results were expressed as mean ± SD, with range. Results The mean plasma VEGF level in patients with Behçet's syndrome (291.9 ± 97.1 pg/mL; range 121–532 pg/mL) was higher than that in control subjects (103.0 ± 43.6 pg/mL; range 25–187 pg/mL) and the difference was significant (P < 0.001). Patients with active disease had significantly (P < 0.001) higher VEGF levels than patients with inactive disease (347.6 ± 87.1 vs. 219.9 ± 51.6 pg/mL). In addition, ocular Behçet's patients (n = 23) had higher VEGF levels (315.7 ± 92.1 pg/mL) than nonocular patients (n = 16, 257.8 ± 96.6 pg/mL) and the difference was of borderline significance (P = 0.041). The levels of all acute‐phase reactant parameters were significantly higher in the active stage than in the inactive stage (for each, P < 0.01) or in control subjects (for each, P < 0.001). Conclusions VEGF may participate in the course of Behçet's syndrome, especially in the active stage, and elevated levels of VEGF may be an additional risk factor for the development of ocular disease, contributing to poor visual outcome.     

Caspase-9 expression is increased in endothelial cells of active Behçet's disease patients

Background Behçet's disease is a multisystem disease of unknown etiology. Caspase‐9 is responsible for initiating the caspase activation cascade during apoptosis. The aim of this study was to examine caspase‐9 expression in both endothelial and perivascular infiltrates of patients with active Behçet's disease. Methods Fifteen patients with active Behçet's disease, attending the First Dermatology Department, Ankara Numune Hospital, Ankara, Turkey between June 2003 and December 2005, were included in the study. Oral biopsy specimens from nine healthy volunteers were taken as the healthy control group, and skin biopsies from 18 psoriasis patients were used as the inflammatory control group. The specimens were examined with caspase‐9 primary antibody. Statistical analyses were performed using SPSS 11.5. Results The mean caspase‐9‐positive endothelial cell counts were 7.17 ± 2.45 in active Behçet's disease, 4.81 ± 0.76 in healthy controls, and 4.35 ± 1.34 in inflammatory controls. The difference between Behçet's disease and healthy controls was statistically significant, with increased endothelial staining in active Behçet's disease (P = 0.049). The difference between Behçet's disease and inflammatory controls was also statistically significant; the rate of staining was higher in Behçet's disease (P = 0.006). The mean caspase‐9‐positive dermal perivascular cell counts were 5.15 ± 2.32 in Behçet's disease, 3.32 ± 0.82 in healthy controls, and 5.54 ± 4.95 in inflammatory controls. These values did not show any statistically significant difference (P = 0.407). Conclusion Endothelial cells are one of the key cells in Behçet's disease, and our findings support the role of endothelial cells in the etiopathogenesis of Behçet's disease.     

Formation of arcuate crusted lesions after non-ablative 1450-nm diode laser treatment equipped with a dynamic cooling device

Abstract

Behçet's disease (BD) is a chronic systemic inflammatory disorder of unknown etiology with variable clinical manifestations. HLA-B51 allele is the most strongly associated known genetic factor. The mucocutaneous lesions (oral aphthae, genital aphthae, skin lesions such as pseudofolliculitis) constitute the hallmark of the disease, but also gastrointestinal, vascular, central nervous systems, and others may be involved. We report a case of a young man affected with Behçet's disease who presented facial telangiectasias and striae rubra in the inner region of his arms and at the level of his hips, as uncommon minor superficial vascular manifestations of BD. To manage them we have subjected the patient to a cycle of Intense Pulsed Light (IPL) therapy. Our findings showed that the use of IPL is a safe and effective treatment for telangiectasias and striae rubra, also in the complex clinical condition of Behçet's disease. In fact, the treatments were well tolerated, no sign of scarring or hyper/hypopigmentation was reported and we obtained a significant improvement of the lesions in terms of color and size of them.     

Clinical features of Behçet's disease in children

Background Behçet's disease (BD) is a systemic inflammatory vasculitis of young adults with unknown etiology. Objective The aim of this study was to determine the clinical features and outcome of BD in childhood. Patients and methods Seventeen patients with BD within a pediatric age group were studied since 1997. Results Boys : girls ratio was 12:5. Familial cases were very frequent (45%). Oral aphthous ulcers were present in all patients (100%), genital aphthous ulcers were present in 16 (94%), ocular lesions in 4 (24%), erythema nodosum in 3 (18%), papulopustules in 8 (47%), joint symptoms in 13 (76%), mild gastrointestinal symptoms in 2 (12%), neurological involvement in 2 (12%), positive pathergy reactions in 13 (76%), and thrombophlebitis in 1 (6%). We used colchicine, systemic corticosteroids, cyclosporine, nonsteroidal anti‐inflammatory drugs, sulfasalazine, and topical agents for the treatment of children with BD. Conclusion The findings of BD in children are similar as in adults, but the frequency of familial cases is significantly higher.     

Clinical evaluation of Behçet's disease: a five year follow-up study

The frequencies of clinical features of Behçet's disease vary in different studies according to the diagnostic criteria used in the study, the department where the study is done, and the ethnic origin of patients included in the study. In this study, we aimed to evaluate the influence of the length of the follow‐up period of patients on the frequencies of clinical features of Behçet's disease. Out of 231 patients who applied to our dermatology clinic from June of 1991 to October of 1996, only 52 patients that were followed up for at least five years were included in this retrospective study. The baseline characteristics of these 52 patients were almost the same as those of the 179 patients who dropped out. Almost all of the frequencies of clinical features at the end of the follow‐up period of five years were higher than those at the time of presentation. The most prominent increases were observed for erythema nodosum (59.6% to 75.0%), papulopustular lesions (84.6% to 94.2%), and large vessel involvement (23.1% to 30.8%). According to our results, it is most likely that studies mainly including mostly patients with long‐standing disease will report higher figures for the frequencies of these clinical features of Behçet's disease.