Behçet’s disease in concurrence with psoriasis

Background Behçet’s disease (BD) and psoriasis are chronic inflammatory diseases characterized by multisystemic vasculitis and epidermal hyperplasia respectively. Although it has been found that the pathogenesis of BD and psoriasis share common perspectives, reports of patients who have both diseases in concurrence are rare. Objectives To analyse and evaluate the clinical manifestations of BD patients who have psoriasis together. Methods Retrospective evaluation of the medical records of nine BD patients who were also diagnosed with psoriasis at the BD Specialty Clinic of Severance Hospital was carried out. We analysed the characteristics of patients and the clinical activity of both diseases, and also the effect of the treatment of one disease against the other. Results Of the nine BD patients who also had psoriasis, male to female ratio was 1 : 2. Two (22.2%) patients had a complete type of BD and seven (77.8%) patients had an incomplete type of BD. For the psoriatic lesions, all nine (100%) patients were diagnosed as psoriasis vulgaris. Five (55.6%) patients had BD as the preceding disease and four (44.4%) patients had psoriasis as the preceding. All five patients who formerly developed BD followed by psoriasis had an active state of BD, but the activity of psoriasis of all nine patients was minimal to average. Conclusion In this study, we evaluated the clinical manifestations of nine patients who had BD and psoriasis together. Although the exact pathogenesis remains unclear, there might be some influence by each disease to the other between BD and psoriasis.     

Cardiac findings in Behçet’s patients

Behçet’s disease is a chronic multi‐system inflammatory disorder and the severity and clinical manifestations of Behçet’s patients may show geographic variation. We aimed to detect the cardiac findings in 30 Behçet’s patients and compare them with the normal population (n = 29). We used color‐doppler echocardiography and transesophageal echocardiography in combination. We calculated manually QT intervals and QT dispersion (QTd) from twelve‐lead ECG recordings. There was no E/A inversion and coronary ischemia in all patients or control group. The E velocity difference between groups was not significant. The mean A velocity was significantly lower in Behçet’s patients than normal group. The mean DT was 154.4 ± 5.8 msec in Behçet’s patients and 122.59 ± 0.96 msec in control group (P < 0.0001). The mean IVRT was 75.66 ± 1.36 msec in Behçet’s patients and 69.1 ± 0.55 msec in control group (P < 0.0001). There was no QTc time difference between the Behçet’s patients and the control group. The mean QT dispersion (QTd) interval was 45.46 ± 2.65 msec in Behçet’s patients and 31.83 ± 1.23 msec in control group (P < 0.0001). Atrial septal aneurysm, mitral valve prolapse and insufficiency, tricuspid valve insufficieny, and pulmonary hypertension frequencies in Behçet’s patients were significantly higher than in the control group. We concluded that Behçet’s cardiac involvement may effect cardiac structure and cause diastolic dysfunction, electrical instability and structural abnormalities. We also concluded that cardiac involvement in Behçet’s disease may be specific for this geographic area.     

Reduced erythrocyte deformability in active and untreated Behçet’s disease patients

Objective Although the etiology of Behcet’s disease is not clarified yet, the vascular endothelial damage and thrombosis are prominent features of the disease. The underlying mechanisms of increased risk of thrombosis in Behcet’s disease are not completely understood. It is stated that the changes in blood rheology such as erythrocyte deformability take part in the thrombosis. There are limited numbers of studies with conflicting results about the erythrocyte deformability in Behcet’s disease. The previous studies were not limited to the untreated patients with active disease. Method Blood samples were filtered immediately by “Imugard‐ leukocyte removal filter”. After plasma and erythrocytes were separated by centrifugation, 10% erythrocyte suspensions were prepared with isotonic Tris NaCl buffer solution. These erythrocyte suspensions were filtered under gravitational force by “Nucleopore” to determine the filtration time. The filtration times of the erythrocyte and buffer solutions were referred as t1 and t2 respectively. Finally, deformability indices were obtained by dividing the t1 by t2. A decrease in the deformability of the erythrocytes would result in an increase in the filtration time of the erythrocyte suspension and deformability indices. Results The median deformability indices were 3.090 and 2.170 for patients and control subjects respectively and the difference was statistically significant (P = 0.000). Conclusion We suggest that the decrease in erythrocyte deformability in Behcet’s disease may be related to oxidative damage of erythrocytes and subsequently takes part in the development of thrombosis in Behcet’s disease.     

The spectrum of mucocutaneous manifestations in Adamantiades‐Behçet’s disease in Greece

Background This is the largest specific demographic and clinical study performed until now in Greece. Objectives To analyse the spectrum of mucocutaneous manifestations in 202 patients with Adamantiades‐Behçet’s disease (ABD) in Greece. Methods Any mucocutaneous symptom at disease onset and during the follow‐up was recorded in a particular questionnaire that included 58 items. All patients fulfilled the International Study Group Criteria for BD. Results Consecutive patients (130 men and 72 women) were included in this study. Their mean age was 42.03 ± 12.41 and 44.96 ± 11.99 years for male and female patients respectively. Pathergy test was positive in 38%, whereas HLA‐B5 (51) positivity was evident in 76% of patients. Onset signs: oral aphthous ulcers were found in 64.36%, genital ulcers in 6.93%, skin lesions in 8.91%, erythema nodosum in 7.42% and pseudofolliculitis in 1.5%. One patient had leg ulcers. During the follow‐up, oral aphthous ulcers were found in 100%, genital ulcers in 65.4% and in 51.4%, erythema nodosum in 42.9% and in 78.1%, whereas pseudofolliculitis in 57.1% and in 21.9% in men and women respectively. Significant differences pointing to a different course of mucocutaneous disease were found between men and women. Conclusions The nature and frequency of mucocutaneous manifestations at presentation are important for the diagnosis of ABD, whereas significant differences were found between genders in this Greek patient cohort. Significant differences were also observed when our results were compared with those of four other series, probably explained by genetic and environmental factors.     

Behçet’s disease and vitiligo in two brothers: coincidence or association?

Behçet’s disease (BD) and vitiligo are disorders with unknown aetiology. We report on two brothers with ocular Behcet’s disease who had advanced unilateral ophthalmic symptoms associated with vitiligo. The two brothers had recurrent oral and genital ulceration, uveitis and white patches on their skin. The most probable hypothesis for the aetiology of BD is that of an autoimmune reaction in genetically predisposed individuals, with vasculitis as the main pathological hallmark. Despite many years of research, the specific causes of vitiligo remain obscure, and the most advanced aetiological hypothesis remains that of autoimmunity. To our knowledge, this is the first reported case within the literature of BD associated with vitiligo. The existence of the two different disorders is noteworthy as they were observed in two brothers during the same period in their lives, with very similar clinical observations.     

Lipoprotein‐associated phospholipase A2 level in patients with Behçet’s disease

Background Behçet’s disease (BD) is a chronic multisystem inflammatory disorder characterized by vasculitis. Vasculitis is thought to underlie many of the clinical manifestations of Behçet’s disease. Lipoprotein‐associated phospholipase A₂ (Lp‐PLA₂) is a highly specific biomarker for vascular inflammation, and has low biological variability. Those features make it more attractive than other inflammatory markers including C‐reactive protein (CRP) and erythrocyte sedimentation rate (ESR), which may reflect systemic inflammation non‐specifically. Objectives It was aimed to investigate circulating Lp‐PLA₂ levels and its relationship with CRP and ESR in patients with BD by considering disease activity. Methods Study group included 72 patients with BD (34 men and 38 women with a mean age of 35.3 years) and 30 sex‐ and age‐matched healthy subjects (15 men and 15 women with a mean age 32.6 years). Patients group included 40 patients with active and 32 patients with inactive BD. Results Lp‐PLA₂, CRP and ESR levels were found to be significantly higher in patient group than controls. In addition, those levels were also significantly higher in patients with active BD than in patients with inactive disease. Lp‐PLA₂ showed positive correlations with CRP and ESR (r = 0.63, P < 0.05 and r = 0.33, P < 0.05 respectively). Lp‐PLA₂ also showed significant important area under curve (AUC) value (0.779), besides CRP (0.941) and ESR (0.888). Optimum cut‐off value was obtained as 218.5 ng/mL. Conclusions It was concluded that Lp‐PLA₂ may be a new useful biomarker to evaluate clinical or subclinical activity of the disease besides CRP and ESR.     

Evaluation of sexual function in patients presenting with Behçet’s disease with or without depression

Aim Sexual dysfunction has been found in many disorders that are chronic or disabling. The aim of this study was to evaluate the sexual satisfaction levels, sexual function and their relationship with the mental state in a group of patients being followed‐up with a diagnosis of Behçet’s disease (BD). Method A total of 50 BD patients and 50 control‐group subjects were administered the Hamilton Depression Rating Scale (HDRS), Hamilton Anxiety Rating Scale (HARS), Golombok Rust Sexual Satisfaction Scale (GRISS) and Arizona Sexual Experiences Scale (ASEX). Results The ASEX, GRISS total, HDRS and HARS scores were significantly higher in the patient group than the control subjects (P = 0.0001, P = 0.007, P = 0.0001, P = 0.0001 respectively). Sexual dissatisfaction was seen in 40 (80%) of the patient‐group and 16 (32%) of the control‐group subjects according to the GRISS (P = 0.0001). Female study participants had higher mean scores than the control subjects for the ASEX, GRISS total scores and the GRISS satisfaction, avoidance, vaginismus and orgasm subscale scores (P = 0.0001, P = 0.002, P = 0.02, P = 0.001, P = 0.006, P = 0.03 respectively). Male study participants had different mean scores for the controls regarding the ASEX scores and the GRISS impotence, premature ejaculation, satisfaction and frequency subscale scores (P = 0.01, P = 0.01, P = 0.0001, P = 0.03, P = 0.007 respectively). Discussion The negative effect of the disorder on the biological and functional status and daily living activities in BD patients also influences the patients’ sexual experiences and satisfaction. The negative effects of chronic diseases such as BD should therefore be defined and the disorder evaluated from a wide perspective during the treatment process.     

Salivary levels of HNP 1–3 are related to oral ulcer activity in Behçet’s disease

Background Saliva contains antimicrobial peptides derived from oral epithelium as well as neutrophils in the innate immune response. The aim of this study was to examine the association between salivary human neutrophil peptide (HNP) 1–3 levels originating from neutrophils and oral ulcers in patients with Behçet’s disease (BD). Methods Ninety‐five patients with BD (F/M: 39/56; mean age: 38.7 ± 11.9 years) and 53 healthy controls (HC; F/M: 23/30; mean age: 35.2 ± 10.1 years) were included in the study. The disease control group (F/M: 20/33; mean age: 33.7 ± 10.7 years) was comprised of patients with oral infection regarding endodontic infection (n = 32) and pericoronitis (n = 21). Salivary HNP 1–3 levels of groups were measured in unstimulated samples by ELISA (Hycult, the Netherlands). Results A statistically significant increase was found in salivary HNP 1–3 levels of patients with BD (2268.28 ± 1216.38 μg/ml) compared with HC (1836.49 ± 857.76 μg/ml), patients with endodontic infection (849.9 ± 376.1 μg/ml), and patients with pericoronitis (824.3 ± 284.02 μg/ml; P = 0.024, 0.000 and 0.000, respectively). The ratio of active oral ulcer (100%, n = 14) was higher in low HNP 1–3 levels (≤1000 μg/ml) than the others (66.7%, n = 54) in active patients with BD (P = 0.008). Moreover, salivary HNP 1–3 levels were significantly lower in patients with endodontic infection and patients with pericoronitis compared with those in the HC group and patients with BD (P = 0.000). Conclusion A decrease in salivary HNP 1–3 levels might be a biological factor for predisposition to oral ulcers in patients with BD and oral infection in healthy patients.     

A randomized,controlled and blinded study of papulopustular lesions in Turkish Behçet’s patients

Background Papulopustular lesions (PPL), the commonest presentation of skin lesions in Behçet’s disease (BD) are cutaneous, sterile folliculitis or acne-like lesions on erythematous base. Our purpose was to determine the true frequency and anatomic location of the PPL and compare this with controls. We also sought to determine whether or not there was any relationship between PPL and either disease activity or other manifestations of BD. Methods Fifty patients with BD, diagnosed according to the criteria of the International Study Group for Behçet’s Disease, were enrolled in the study. The control group consisted of 100 patients with other dermatologic diseases (21 acne and 79 non-acne patients), selected randomly. A dermatologist counted the lesions, in a blind protocol, on seven anatomic locations: scalp, face, neck, trunk, upper and lower extremities and genitalia. Results The frequency of PPL in patients with BD was 96% and the most common location was the trunk, whereas in the control group the frequency was 89% and the most common location was the face. In acne and non-acne patients, the frequency was 100% and 86.1% respectively. The total mean number, and mean numbers of PPL on the location of trunk, upper and lower extremities, and genitalia were higher in patients with BD than in controls. When the PPL in BD patients with a positive pathergy test was compared with that in patients with a negative pathergy test, the difference was significantly higher. Conclusions Our results indicate that PPL appear to be non-specific. In the diagnosis of BD the mean number and anatomic location of the lesions are of more importance than the frequency.     

Increased arterial stiffness assessed by pulse wave velocity in Behçet’s disease and its association with the lipid profile

Objective To evaluate the structural and functional properties of vessels in Behçet’s Disease (BD) using carotid‐femoral pulse wave velocity (PWV) and an echo‐tracking system. Methods BD patients without traditional cardiovascular risk factors were selected. All BD patients performed PWV and carotid ultrasound. BD patients were divided into groups based on the presence of systemic (vascular and/or ocular and/or central nervous system involvement) and vascular involvement. Healthy controls age‐ and sex‐matched with the same exclusion criteria were selected. Results A total of 23 BD patients (mean age 35.0 ± 7.6 years) had significantly higher PWV levels compared with controls (8.48 ± 1.14 vs. 7.53 ± 1.40 m/s, P = 0.017). Intima‐media thickness (594.87 ± 138.61 vs. 561.08 ± 134.26 μm, P = 0.371), diastolic diameter (6383.78 ± 960.49 vs. 6447.65 ± 1159.73 μm, P = 0.840), distension (401.95 ± 117.72 vs. 337.91 ± 175.36 μm, P = 0.225) and relative distension (6.26 ± 2.83 vs. 5.42 ± 2.46 μm, P = 0.293) were similar in both groups. The systemic disease group had significantly higher levels of PWV (8.79 ± 1.21 vs. 7.88 ± 0.72 m/s, P = 0.036) compared to those with exclusive mucocutaneous manifestations. BD patients with vascular involvement had similar PWV and echo‐tracking parameters compared to those without vascular involvement (P > 0.05), but had higher total and LDL cholesterol levels (P = 0.019 and P = 0.012, respectively). The multivariate linear regression analysis identified triglycerides as the most important factor in increasing PWV levels (P = 0.001) in BD. Conclusions PWV is more useful than carotid ultrasound in detecting structural and functional vascular damage in BD and emphasizes the role of the disease itself in promoting these alterations. Our findings also reinforce the need for rigorous control of all risk factors in BD, particularly lipoproteins.     

Adiponectin levels,insulin resistance and their relationship with serum levels of inflammatory cytokines in patients with Behçet’s disease

Aim Increased frequency of cardiovascular disease and its possible relations with insulin resistance have been reported in patients with inflammatory diseases. The aim of our study was to investigate insulin resistance and serum adiponectin levels as cardiovascular risk markers in patients with Behçet’s disease. Method Study population consisted of 40 patients with Behçet’s disease (BD) and a control group composed of age, gender, body mass index‐matched 46 healthy individuals. All patients were examined for signs of Behçet’s disease. Body mass index, waist and hip circumference were measured. Insulin resistance was evaluated using the homeostasis model assessment‐insulin resistance method. Erythrocyte sedimentation rate (ESR), lipid profile, high sensitive CRP (hsCRP), adiponectin, TNF‐α, IL‐6 and IL‐8 levels were measured. Results Erythrocyte sedimentation rate, serum hsCRP and IL‐6 levels were significantly higher in patients with BD than those in the controls (P = 0.001, P = 0.001, P = 0.001, respectively). Fasting plasma glucose, insulin levels and lipid profile were not different between the two groups. Insulin resistance and decreased levels of the serum adiponectin were not detected in the patients. There was no relationship between insulin resistance, adiponectin levels and inflammatory markers. Active and inactive patients did not differ in respect of any parameters. Conclusion Being a systemic vasculitis, BD may cause cardiovascular involvement. In this study, dyslipidemia, insulin resistance and low adiponectin levels were not detected among our patients with Behçet’s disease. Our results suggest that there exists no increased risk for atherosclerotic cardiovascular disease associated with adiponectin levels and insulin resistance in patients with Behçet’s disease.     

Serum galectin‐3 levels in patients with Behçet’s disease: association with disease activity over a long‐term follow‐up

Background There is a need for a laboratory marker that correlates with the clinical activity of Behçet’s disease (BD). Objective We aimed to investigate whether serum galectin‐3 (Gal‐3) levels were affected during the course of the disease with regard to disease activity. Methods A total of 131 subjects were involved in the study as follows: Group 1: BD active (n = 39); Group 2: BD inactive (n = 31); Group 3: Disease controls with leucocytoclastic vasculitis confirmed with a skin biopsy (n = 22); and Group 4: Healthy control subjects (n = 39). The BD patients were followed regularly and samples were taken in their active and inactive periods of the disease over a 2‐year period. Results Serum Gal‐3 levels were significantly higher in active BD patients (mean 2.38) than inactive BD patients (mean 0.63; P < 0.0001) and the healthy control subjects (mean 0.75; P < 0.0001). There was no significant difference between the leucocytoclastic vasculitis and active BD patients (P = 0.093). Serum Gal‐3 levels were positively correlated with clinical activity scores of active BD patients (r = 0.66, P < 0.0001). In addition, the Gal‐3 levels were significantly higher in the active disease period when compared with the inactive period during the follow‐up. There were no significant differences between the two inactive periods of the disease among the same patients. Further analyses revealed that patients with vascular involvement had significantly higher Gal‐3 levels than the other active BD patients (mean 7.57; P = 0.007). Limitations The limitation of the study is the small number of patients with vascular involvement in the active BD patient group. Conclusion Gal‐3 levels are correlated with the activity of Behçet’s disease especially with the vascular involvement.     

Angiokeratoma: a cutaneous marker of Fabry’s disease

The initial symptoms of Fabry’s disease (FD) may seem harmless and may delay its diagnosis. A survey and screening for FD were performed on men with biopsy‐proven angiokeratoma and some of their relatives (n = 29). Three patients were identified. Dermatologists should be aware of this prominent early feature and investigate unexplained cutaneous vascular lesions to detect FD.     

Primary cutaneous Hodgkin’s lymphoma

Cutaneous involvement in Hodgkin’s lymphoma is an uncommon but well‐recognized condition that usually occurs with advanced‐stage disease. Primary cutaneous Hodgkin’s disease (PCHD) is exceedingly rare, with only a few reported cases. We report a case of a man treated with combination systemic chemotherapy for PCHD, and review the available literature.