Brain perfusion MRI findings in patients with Behcet's disease.

OBJECTIVE: To search brain perfusion MRI (pMRI) changes in Behcet's disease (BD) with or without neurological involvement. MATERIALS AND METHOD: The pMRI were performed in 34 patients with BD and 16 healthy controls. Based on neurologic examination and post-contrast MRI, 12 patients were classified as Neuro-Behcet (group 1, NBD) and 22 patients as BD without neurological involvement (group 2). Mean transit time (MTT), time to peak (TTP), relative cerebral blood volume (rCBV), and relative cerebral blood flow (rCBF) were obtained and compared to those of healthy control group (group 3). RESULTS: There was a significant difference in the MTT and rCBF within the pons and parietal cortex in groups 1 and 2. rCBV increased in cerebral pedicle in group 1 compared with groups 2 and 3. In the temporal lobe white matter, prolonged MTT and decreased rCBF were found in groups 1 and 2. In the corpus striatum, internal capsule, and periventricular white matter, rCBF increased in group 1 compared with group 3 and decreased in groups 1 and 2. CONCLUSION: Brain pMRI is a very sensitive method to detect brain involvement in patients with BD and aids the clinical diagnosis of NBD, especially in patients with negative MRI findings.

Subclinical left ventricular dysfunction in Behcet's disease assessed by two-dimensional speckle tracking echocardiography.

AIMS: The aim of this study was to evaluate the left ventricular (LV) systolic strain by speckle tracking echocardiography (STE) in order to provide the early detection of myocardial dysfunction in patients with Behcet's disease (BD). We also aimed to examine the relationship between LV systolic strain and N-terminal pro-B type natriuretic peptide (NT-proBNP), which is a cardiac biomarker of ventricular dysfunction. METHODS AND RESULTS: Longitudinal and circumferential systolic strain assessed by STE was obtained in 32 BD patients and 27 age-matched controls. NT-proBNP levels were also measured in all subjects. Regional and mean longitudinal strain (-17.8 ± 2.7 vs. -20.5 ± 1.8%; P < 0.0001) was significantly lower in BD patients when compared with the healthy controls. Whereas regional and mean circumferential strain values (-22.0 ± 1.6 vs. -22.2 ± 2.3%; P = 0.62) did not reveal a significant difference between the patients and the controls. NT-proBNP was significantly higher in the patients than in the controls (65.18 ± 84.51 vs. 30.84 ± 14.75 pg/mL; P = 0.003). Linear regression analyses revealed only NT-proBNP as the independent correlate of mean LV longitudinal strain (R = 0.603, P = 0.001). CONCLUSION: Longitudinal myocardial systolic function assessed by STE, which is a sensitive marker of subclinical ventricular dysfunction is impaired in BD. Increased NT-proBNP levels may be a sign of subclinical ventricular dysfunction in these patients.

Evaluation of 60 patients with pilonidal sinus treated with laser epilation after surgery.

BACKGROUND: The surgical treatments for pilonidal sinus disease often result in recurrences, and the patients risk requiring multiple surgical interventions. OBJECTIVE: To evaluate the role of alexandrite laser hair removal after surgery in pilonidal sinus disease. METHODS: Sixty patients who underwent surgical treatment of pilonidal sinus disease and were treated with a 755-nm alexandrite laser after surgery between 1999 and 2007 were examined retrospectively. The charts were reviewed, and the patients were interviewed on the telephone about their post-laser period and recurrence. The laser parameters, patient history, and surgical details were recorded. RESULTS: The overall recurrence rate was 13.3%, after a mean follow-up period +/- standard error of the mean of 4.8 +/- 0.3 years. The mean number of laser treatment was 2.7 +/- 0.1. Seventy-five percent of the recurrences were detected after a follow-up period of 5 to 9 years. Fifty percent of the recurrent cases had drainage and secondary intention before the laser epilation. CONCLUSION: Our results strongly suggest that laser hair removal after surgical interventions in pilonidal sinus disease decreases the risk of recurrence over the long term.

Pulsed dye laser in the treatment of nail psoriasis.

BACKGROUND: The treatment options for nail psoriasis have been limited, and the management of nail psoriasis has been challenging for physicians. OBJECTIVES: To evaluate the effect of pulsed dye laser (PDL) in the treatment of nail psoriasis. METHODS: Psoriatic nails of five patients were treated using PDL (595 nm) once monthly for 3 months. The pulse duration was 1.5 ms, the beam diameter was 7 mm, and the laser energy was 8.0 to 10.0 J/cm(2). Clinical efficacy was statistically evaluated according to Nail Psoriasis Severity Index (NAPSI) score differences before and after the treatment. RESULTS: Statistical analysis of NAPSI scores before and after treatment showed significant difference (p<.05, paired t-test). The nail bed lesions, particularly onycholysis and subungual hyperkeratosis, responded best to the treatment. LIMITATIONS: Limitations include the lack of blinding and comparison and the small number of patients. CONCLUSION: PDL might be an alternative treatment for nail psoriasis. The authors have indicated no significant interest with commercial supporters.

Increased mean platelet volume in Behçet's disease with thrombotic tendency.

The relationship between Behçet's disease (BD) and platelet aggregation has not sufficiently been investigated yet. Mean platelet volume (MPV) is a marker of platelet function, and the increase in MPV has been identified as an independent risk factor of recurrent vascular events. BD is characterized by a relapsing vasculitis of the venous as well as arterial thrombosis. However, the precise pathogenic mechanisms underlying thrombotic tendency in BD are not known. We hypothesized that there might be an association between thrombotic complication and MPV in these patients. Therefore, we investigated activation of platelets in patients with BD using a simple marker, MPV, the most accurate measure of platelet size. A total of 60 patients with BD and 40 age- and gender-matched controls were included. The BD patients were divided into subgroups based on the presence (n = 22) or absence of thrombosis (n = 38) and clinically active (n = 30) or inactive (n = 30) state. MPV was higher in patients with BD than controls (8.14 +/- 0.8 vs. 7.48 +/- 0.3 fl, p = 0.001). Among BD patients, MPV was larger in patients with thrombosis than those without thrombosis (8.45 +/- 1.0 vs. 7.96 +/- 0.7 fl, p = 0.038). However, there was no significant difference in MPV between BD patients with active and inactive states. The increase in MPV is independent of the disease activity, and the presence of thrombosis is associated with higher MPV in BD patients. Therefore, antiplatelet therapy may be useful to prevent thrombotic complications in BD patients.

Vitiligo koebnerized by behçet disease genital ulceration.

A 50-year-old man was diagnosed with Behçet disease (BD) on the basis of an 8-year history of episodes of recurrent oral aphthous ulcers, papulopustular lesions on physical examination, and a positive pathergy test. Two years prior to diagnosis of BD, vitiligo had also been diagnosed on the basis of hypopigmented lesions on the scrotum following genital ulceration. To our knowledge, this is the first case of incidental coexistence of BD and vitiligo, and, furthermore, the first case of koebnerization of genital ulceration of BD.

Incontinentia pigmenti: a case report and literature review.

Incontinentia pigmenti (IP) is a rare, X-linked dominant disorder that presents at or soon after birth with characteristic cutaneous signs. The eyes and central nervous system are the next most commonly affected systems. We aimed to describe the ophthalmological, neurological and radiodiagnostic findings of a patient with IP and bilateral retinal detachment. Clinical and laboratory findings of a four-month-old female baby who did not have light fixation and had neurological maturation retardation are presented. Characteristic skin lesions of IP were noted especially at the extremities, bilaterally. On neurological examination, motor and mental maturation were retarded and axial hypotonia was noted. Bilateral retinal detachment was the cause of absent eye fixation noted during ophthalmologic examination, and the detachments were also documented by ultrasonography and magnetic resonance imaging (MRI). Otologic examination was normal. Focal left frontal lobe atrophy, corpus callosum hypoplasia and prominence of right hemisphere were also noted on MRI. MR spectroscopy revealed negative lactate peak at the involved left frontal lobe. Bilateral retinal detachment is a probable finding in IP and patients with neurological symptoms should be investigated for associated sight-threatening ocular pathologies.

Demographic and clinical properties of juvenile-onset Behçet's disease: A controlled multicenter study.

BACKGROUND: Behçet's disease (BD) is a multisystemic inflammatory disorder of unknown origin. The disease usually occurs between the second and the fourth decades, whereas it is uncommon in children. OBJECTIVE: In this multicenter study, we aimed to describe the demographic and clinical features along with severity in juvenile- versus adult-onset BD. METHODS: Patients with initial symptoms at age 16 years or younger were considered as having juvenile-onset BD. In all, 83 patients with juvenile-onset BD (38 male and 45 female; mean age 19.6 +/- 7.6 years) and 536 with adult-onset (>16 years) BD (293 male and 243 female; mean age 39.2 +/- 10.1 years) who fulfilled the classification criteria of the International Study Group for BD were involved in the study. RESULTS: Familial cases were more frequent in juvenile-onset compared with adult-onset BD (19% vs 10.3%; P = .017). The mean age of disease onset was 12.29 +/- 3.54 years in juvenile-onset BD and 31.66 +/- 8.71 years in adult-onset BD. Mucocutaneous lesions and articular symptoms were the most commonly observed manifestations in both groups. The frequency of disease manifestations was not different between juvenile- and adult-onset BD, except neurologic and gastrointestinal involvement, which were higher in juvenile-onset BD than adult-onset BD (P = .027 and P = .024, respectively). Oral ulcer was the most common onset manifestation of both juvenile-onset (86.74%) and adult-onset (89.55%) BD. The frequencies of onset manifestations of BD were similar, except genital ulcer, which was higher in adult-onset BD (P = .025). LIMITATIONS: Our study consisted of patients with BD mainly applying to dermatology and venerology departments. Therefore, it can be speculated that this study includes rather a milder spectrum of the disease. CONCLUSIONS: Although the clinical spectrum of juvenile-onset BD seems to be similar to adult-onset BD, the frequency of severe organ involvement was higher. Because of the higher prevalence of familial cases in juvenile-onset BD, it can be speculated that genetic factors may favor early expression of the disease with severe organ involvement.

Relationship between serum homocysteine levels and structural-functional carotid arterial abnormalities in inactive Behçet's disease.

BACKGROUND: Behçet's disease (BD) is a chronic autoimmune disorder with symptoms manifesting from an underlying vasculitis. Since the disease activity is correlated with characteristic vascular endothelial dysfunction, BD places individuals at increased risk of cardiovascular diseases, such as atherosclerosis. Hyperhomocysteinaemia is an independent risk factor for arteriosclerotic vascular diseases. AIM: This study was designed to investigate how plasma homocysteine (Hcy) affects the structural and functional properties of the carotid artery in humans. METHODS: Sixty-eight BD patients with subclinical atherosclerosis and 40 healthy controls underwent carotid sonography and Doppler ultrasound to measure carotid artery intima-media thickness (C-IMT) and carotid stiffness and distensibility (indicat-ing elasticity). Total Hcy level was determined by enzyme-linked immunosorbent assay. For analysis, the BD patients were sub-grouped according to hyperhomocysteinaemia (> 15 µmol/L). RESULTS: The patients with BD were found to have increased C-IMT and beta stiffness and decreased distensibility. In addition, hyperhomocysteinaemia was significantly correlated with these detrimental changes in the carotid artery, possibly raising the risk of these patients developing atherosclerosis. CONCLUSIONS: These findings suggest a potential mechanism of atherosclerosis in BD and highlight the processes that future research should focus on to address identification and prophylactic treatment of BD patients at risk of cardiovascular disease.

High prevalence of glucose metabolism disturbance in patients with lichen planus.

AIM: To establish the prevalence of diabetes mellitus (DM) and insulin resistance in patients with lichen planus (LP), and to examine whether diabetic status showed any relation with the type of LP. PATIENTS AND METHODS: Thirty patients with LP and 30 age, weight, and sex matched volunteers were included. Serum fasting glucose, insulin, hemoglobin A1c levels were determined, a standard oral glucose tolerance test (OGTT) was performed. RESULTS: Of patients with LP, eight (26.7%) had DM (four newly diagnosed), however, only one (3.33%) diagnosed as DM in control group (p=0.007). Six patients (20.0%) with LP and four healthy persons (13.3%) had IGT (p>0.05). Glucose metabolism disturbance (DM+IGT) was detected in 14 (46.7%) of the patients and in 5 (16.7%) of the controls (p=0.026). HbA1c, fasting serum glucose, and insulin resistance (HOMA) were statistically higher in patients compared to controls. CONCLUSION: Our finding documented that approximately one half of the patients with LP had glucose metabolism disturbance, and one fourth had DM. We believe that further studies are needed to explain this close relationship between DM and LP.

Scalp ulcer as first sign of cholangiocarcinoma.

Metastases from distant primary tumors are rarely the first indicators of cancer. However, cutaneous metastases are not uncommon in cases of internal malignancy. This article describes the case of a 65-year-old man with cutaneous metastasis of cholangiocarcinoma who presented with a scalp ulcer as the only initial sign. This is only the third reported case of cholangiocarcinoma with distant cutaneous metastasis.

Monilethrix: improvement with acitretin.

Monilethrix is a rare hereditary disorder that affects the hair and is characterized by shaft anomaly. There is no known treatment that successfully cures the condition. In this report we present a case of monilethrix in a 7-year-old girl treated with oral acitretin. A very good clinical and cosmetic result was obtained while treatment was continued. However, clinical symptoms recurred after discontinuation of acitretin therapy.

Development of large genital ulcer due to hydroxyurea treatment in a patient with chronic myeloid leukemia and Behcet's disease.

Chronic myeloid leukemia (CML) is a myeloproliferative disease arising from abnormal stem cells and associated with splenomegaly and leukocytosis. Hydroxyurea and interferon alpha are used in treatment very frequently. Behcet's disease (BD) is a chronic, repetitive disease of unknown etiology and associated with mucocutaneous, ocular, vascular and central nervous system involvement. Cases with symptoms of BD have been reported during interferon alpha treatment of CML. However, similar symptoms due to hydroxyurea treatment have been reported in only one case until now. A 32-year-old female patient under follow up because of CML and BD, developed a large genital ulcer during hydroxyurea treatment. Ulcers due to hydroxyurea treatment and the co-existence of these two diseases has been reported in only one case in the literature while large genital ulcers have not been reported previously.

The clinical importance of demodex folliculorum presenting with nonspecific facial signs and symptoms.

Demodex folliculorum (D. folliculorum), found in the pilosebaceous unit, is the most common ectoparasite of humans. Various clinical forms such as pustular folliculitis, papulopustular scalp eruptions, perioral dermatitis, and blepharitis have been defined, although in general, the disease has been classified into three main groups as "pityriasis folliculitis", "rosacea-like demodicidosis", and granulomatous rosacea-like "demodicidosis gravis". Our aim was to test for the presence of D. folliculorum in pathogenic numbers in patients who came to our clinic with non-specific symptoms such as facial itching with or without erythema, seborrheic dermatitis-like or perioral dermatitis-like lesions, papulopustular lesions, and an acneiform clinical appearance without telengiectasia or flushing. Twenty-eight (87.5%) female and 4 male (12.5%), patients and 33 age-and-sex matched healthy subjects enrolled in this study. D. folliculorum was sought in the lesion sites using the non-invasive method known as the Standardised Skin Surface Biopsy (SSSB). The discovery of more than five parasites in an area of 1 cm2, was evaluated as pathogenic. For treatment, 5% permethrine cream was applied twice daily for 15 to 30 days. The clinical symptoms of the patients were classified into clinical groups and evaluated as facial itching in 2 (6.3%), nonspecific erythema and itching in 21 (65.6%), erythema and pityriasiform squamous lesions in 3 (9.4%), acneiform in 3 (9.4%), papulopustular lesions in 1 (3.1%), granulomatous rosacea-like in 1 (3.1%), and perioral dermatitis-like symptoms in 1 (3.3%), D. folliculorum density was determined as 5>D/cm2 in all clinical lesions. A significant clinical healing and density of D. folliculorum at <=5 D/cm2 was determined in all but two patients after treatment. We consider that D. folliculorum presentation with different symptoms and signs than classical forms is not rare. For this reason, we suggest that it is useful to test for D. folliculorum in patients with non-classical presentations like facial itching, itching accompanied by non-specific erythema, itching and non-specific pityriasiform squamous lesions, and acneiform lesions.

Renal failure in a patient with autosomal dominant polycystic kidney disease and coexisting dermato-polymyositis: first report in the literature.

Autosomal dominant polycystic kidney disease is a multisystem disorder characterized by multiple, bilateral renal cysts and is also associated with cysts in other organs, such as the liver, pancreas, and arachnoid membranes. Dermatomyositis is a disease which mainly involves the skin and muscles, although occasionally other organs are affected. In this report, a 56-year-old male patient with a four-year history of autosomal dominant polycystic kidney disease was presented. Renal failure was exacerbated by a coexisting dermato-polymyositis. Prednisone treatment with hemodialysis improved the situation. This is the first report renal failure in a patient with autosomal dominant polycystic kidney disease and dermato-polymyositis.

Isokinetic Evaluation of Knee Extensor/Flexor Muscle Strength in Behcet's Patients.

Background: Behçet's disease (BD) is an idiopathic, multisystemic, progressive disease. The purpose of this study is to compare the knee flexor and extensor isokinetic muscle strengths of Behcet's patients with that of healthy subjects. Methods: Twenty-five (13 male and 12 female) patients with BD and 25 (15 male and 10 female) healthy individuals were included in the study. Velocities of 90°/sec, 120°/sec, and 150°/sec were used for the isokinetic muscle strength testing. Patients with active inflammatory knee arthritis were excluded. Peak torque (Nm) and peak torque adjusted to body weight (%) were taken into consideration for comparison between study groups. Results: Compared to healthy controls, there was a statistically significant decrease in both the bilateral knee extensor and flexor muscle isokinetic peak torques(Nm) as well as the peak torques adjusted to body weight (%) at velocities of 90°/sec, 120°/sec and 150°/sec in patients with BD (p < 0.05). However, there was no significant difference in the agonist-antagonist ratio of the isokinetic peak torques of knee muscles between the two groups. Conclusion: In light of these findings, we have concluded that both knee flexor and extensor isokinetic muscle strengths are lower in BD. We therefore recommend careful monitoring of patients with BD in terms of muscle strength.

Assessment of atrial conduction time in patients with Behçet's disease.

OBJECTIVE: Behçet's disease is characterized by increased inflammatory activity, and there there might be an increased risk of atrial arrhythmia in patients with this disease. Our study is aimed to evaluate a novel method of measuring atrial electromechanical features expressed as interatrial and intraatrial electromechanical delay by tissue Doppler echocardiography in patients with Behçet's disease. METHODS: We evaluated 57 patients (mean age: 36.3±12.1 years) with Behçet's disease and 34 sex and age matched healthy volunteers (mean age: 38.4±8.6 years) as control group. P-wave dispersion (PWD) was calculated from the 12-lead surface ECG, interatrial and intraatrial electromechanical delay were measured by tissue Doppler imaging and conventional echocardiography. RESULTS: Interatrial electromechanical delay and intraatrial electromechanical delay were prolonged in patients with active Behçet's disease compared with the patients with inactive disease and the controls (p<0.0001, p<0.0001, p=0.013 and p=0.001, respectively). Erythrocyte sedimentation rate and high-sensitivity C-reactive protein values of of patients with active Behçet's were significantly higher than those with inactive Behçet's disease and the controls (p<0.0001 and p<0.0001, respectively). High-sensitivity C-reactive protein and erythrocyte sedimentation rate were correlated with interatrial electromechanical delay in patients with Behçet's disease (r=0.44, p=0.001 and r=0.64, p<0.0001, respectively). CONCLUSIONS: The prolongation of atrial electromechanical conduction might be related with changes in structure and electrophysiological properties of the atrial myocardium or the conduction system in patients with active Behçet's disease.

The ocular surface in the Behçet's disease patient.

OBJECTIVE: Evaluation of the morphological changes in conjunctival surface epithelial cells with impression cytology in Behçet's disease patients. PATIENTS: A total of 89 eyes of 89 subjects were included. These consisted of 56 patients with inactive Behçet's disease (group 1) and a control group of 33 age- and sex-matched subjects (group 2). METHODS: Schirmer-I and break-up time (BUT) tests were used to evaluate the tear film of the subjects. Impression cytology was used to evaluate morphological changes of the conjunctival surface epithelial cells. The results of the 2 groups were compared. RESULTS: The mean Schirmer-I test result was 8.59 ± 4.83 in group 1 and 14.52 ± 6.38 in group 2. There was a statistically significant difference between the 2 groups for this test (p = 0.001). The mean BUT test value was 10.05 ± 3.26 in group 1 and 13.64 ± 4.28 in group 2. There was a statistically significant difference between the 2 groups for this test (p = 0.001). The mean impression cytology score was 1.65 ± 0.90 in group 1 and 1.39 ± 0.96 in group 2. There was no statistically significant difference between the 2 groups when the cytology grade score was compared (p = 0.227). We found no statistically significant difference for age, gender, disease duration, systemic drug use, Schirmer-I, BUT, and impression cytology degrees between the Behçet's disease patients with and without ocular involvement (p > 0.05). CONCLUSIONS: We found that there was a statistically significant difference between Behçet's disease patients and normal subjects regarding the ocular film layer, but no difference regarding conjunctival surface epithelial morphology.

Left atrial volume and function in patients with Behcet's disease assessed by real-time three-dimensional echocardiography.

AIMS: Behçet's disease (BD), a multisystemic inflammatory disorder, has been associated with a number of cardiovascular dysfunctions, including endomyocardial fibrosis of the right heart, atrial fibrillation, ventricular arrhythmias and sudden cardiac death. The incidence and nature of cardiac involvement in BD are not yet clearly documented. Our aim was to evaluate left atrial (LA) volume and functions using real-time three-dimensional echocardiography (RT3DE) in Behcet's patients without any cardiac symptom. METHODS AND RESULTS: The study included 40 BD (16 females, 24 males and mean age of 33±7 years) and 30 healthy (11 females, 19 males and mean age of 35±6 years) subjects. All the patients' demographic parameters such as age, gender, and duration of BD were recorded. All the individuals underwent comprehensive 2D echocardiography examination, and RT3DE was performed to assess LA volumes and mechanical functions. LA maximum volume (Vmax) and before atrial contraction volume (Vpre A), LA active stroke volume and total stroke volumes (TSV), total emptying and active emptying fractions and expansion index were significantly higher in Behcet's disease patients when compared with the controls (P<0.0001 for all). LA passive emptying fraction was significantly lower in the patients with BD than in the controls (41±7 vs. 44±5, P=0.039). There were positive correlations between TSV and high-sensitive C-reactive protein level (r=0.413, P=0.008), TSV, and disease duration (r=0.417, P<0.007). CONCLUSION: Our study has shown that LA mechanical functions and volumes are impaired in BD. These results may be an early form of subclinical cardiac involvement in patients with BD who have no clinical evidence for cardiovascular disease.