Association of NOD1, NOD2, PYDC1 and PYDC2 genes with Behcet's disease susceptibility and clinical manifestations.

Purpose: Behçet's disease (BD) is an autoinflammatory disease with clinical manifestations such as mucocutaneous, ocular, vascular, gastrointestinal, musculoskeletal and central nervous system involvement. Features of innate and adaptive immunity and inflammasome pathways have been claimed in the pathogenesis of BD. We aimed to investigate the roles of NOD1, NOD2, PYDC1 and PYDC2 genes in the genetic predisposition of BD.Materials and Methods: Genetic variations of NOD1 (rs2075820 and rs2075818) and NOD2 (R334Q and R334W) genes were explored in 68 BD patients and 70 controls with PCR-RFLP (polymerase chain reaction-restriction fragment length polymorphism) approach. PYDC1 and PYDC2 gene variants were investigated by Sanger sequencing.Results: The polymorphism of rs2075820 (NOD1 G/A) had a statistically significant difference between the BD and controls, AA genotype was 2.460-fold protective. When compared in terms of cardiovascular involvement in BD patients, AA genotype was increased the risk of cardiovascular involvement 4.286-fold. There was a significant difference between BD and controls in rs2075818 (NOD1 G/C) polymorphism and CC genotype increased the risk of BD by 3.780-fold. In terms of rs2075818 variants, there was a statistically significant difference between BD patients with ocular lesions, joints, cardiovascular and gastrointestinal involvement and controls. There was a significant difference between the patients with joint involvement and controls and the risk increased of 3.310-fold.Conclusion: The data shed new light on the association between polymorphisms of NOD1 gene and BD and clinicial manifestations. However, NOD2, PYDC1 and PYDC2 genes were not associated with BD in the Turkish population.

Patch Test Results to European Baseline Series in Turkey: A Prospective and Multicenter Study.

BACKGROUND: Patch testing with the European Baseline Series (EBS) is an essential diagnostic tool for the assessment of allergic contact dermatitis. OBJECTIVE: The aims of the study were to describe the most common contact allergens identified with patch testing in Turkey and to establish the distribution of contact allergens among different regions in Turkey. PATIENTS AND METHODS: Twelve centers consisting of tertiary health care institutions were included in the study. A total of 1169 patients were patch tested with the EBS from 2015 to 2017. RESULTS: A total of 596 patients (51.0%) had 1 or more positive patch test reactions to the EBS. A total of 30.2% (n = 353) of those tested had clinical relevance. Contact sensitivity was more common in young patients. The most common 10 allergens were nickel sulfate (20.4%), followed by textile dye mix (8.6%), cobalt chloride (8.3%), potassium dichromate (8.0%), p-phenylenediamine base (4.5%), balsam of Peru (4.3%), methylchloroisothiazolinone/methylisothiazolinone (3.8%), thiuram mix (3.6%), fragrance mix I (3.0%), and methylisothiazolinone (2.9%). CONCLUSIONS: This study provides a comprehensive profile of patch test results with the EBS in Turkey. An ongoing decrease in contact allergy to methylchloroisothiazolinone/methylisothiazolinone and methylisothiazolinone was observed. Textile dye mix and p-phenylenediamine are beginning to pose a new risk in Turkey.

Internalized Stigma in Pediatric Psoriasis: A Comparative Multicenter Study.

BACKGROUND: Internalized stigma, adoption of negative attitudes and stereotypes of the society regarding persons' illness, has not been studied previously in pediatric psoriasis patients. OBJECTIVE: We aimed to investigate the internalized stigma in pediatric psoriasis patients and to determine differences according to factors affecting internalized stigma compared to adult psoriasis patients. METHODS: This multicenter, cross-sectional, comparative study included 125 pediatric (55 female, 70 male; mean age±standard deviation [SD], 14.59±2.87 years) and 1,235 adult psoriasis patients (577 female, 658 male; mean age±SD, 43.3±13.7 years). Psoriasis Internalized Stigma Scale (PISS), Dermatology Life Quality Index (DLQI), Perceived Health Status (PHS), and the General Health Questionnaire (GHQ)-12 were the scales used in the study. RESULTS: The mean PISS was 58.48±14.9 in pediatric group. When PISS subscales of groups were compared, the pediatric group had significantly higher stigma resistance (p=0.01) whereas adult group had higher scores of alienation (p=0.01) and stereotype endorsement (p=0.04). There was a strong correlation between mean values of PISS and DLQI (r=0.423, p=0.001). High internalized stigma scores had no relation to either the severity or localization of disease in pediatric group. However, poor PHS (p=0.007) and low-income levels (p=0.03) in both groups, and body mass index (r=0.181, p=0.04) in the pediatric group were related to high PISS scores. CONCLUSION: Internalized stigma in pediatric patients is as high as adults and is related to poor quality of life, general health, and psychological illnesses. Unlike adults, internalized stigma was mainly determined by psoriasis per se, rather than disease severity or involvement of visible body parts, genitalia or folds.

Internalized stigma in psoriasis: A multicenter study.

Internalized stigma is the adoption of negative attitudes and stereotypes of the society regarding a person's illness. It causes decreased self-esteem and life-satisfaction, increased depression and suicidality, and difficulty in coping with the illness. The primary aim of this study was to investigate the internalized stigma state of psoriatic patients and to identify the factors influencing internalized stigma. The secondary aim was to identify the correlation of internalized stigma with quality of life and perceived health status. This multicentre, cross-sectional study comprised 1485 patients. There was a significant positive correlation between mean values of Psoriasis Internalized Stigma Scale (PISS) and Psoriasis Area and Severity Index, Body Surface Area, Dermatological Life Quality Index and General Health Questionnaire-12 (P < 0.001 in all). Lower percieved health score (P = 0.001), early onset psoriasis (P = 0.016), family history of psoriasis (P = 0.0034), being illiterate (P < 0.001) and lower income level (P < 0.001) were determinants of high PISS scores. Mean PISS values were higher in erythrodermic and generalized pustular psoriasis. Involvement of scalp, face, hand, genitalia and finger nails as well as arthropathic and inverse psoriasis were also related to significantly higher PISS scores (P = 0.001). Our findings imply that psoriatic patients experience high levels of internalized stigma which are associated with psoriasis severity, involvement of visible body parts, genital area, folds or joints, poorer quality of life, negative perceptions of general health and psychological illnesses. Therefore, internalized stigma may be one of the major factors responsible from psychosocial burden of the disease.

Cytomorphometric Characteristics of Buccal Mucosal Cells in Behçet's Disease Patients.

BACKGROUND: The aim of this study was to compare the cytomorphometric characteristics of the buccal cells of Behçet's disease patients with those of healthy controls. METHODS: This case-control study compared a group of 30 patients with Behçet's disease with an age- and gender-matched control group of 30 healthy individuals. The buccal mucosal smears were stained using the Papanicolaou technique for cytomorphometric analyses. The nuclear and cytoplasmic areas were evaluated using digital image analysis; the ratio of nuclear to cytoplasmic areas and nuclear roundness are presented. RESULTS: The nuclear and cytoplasmic areas of the BD patients' cells were significantly smaller than those of the healthy controls' cells, while the nucleus-to-cytoplasm ratio and neutrophil infiltration rate did not differ significantly between the groups. However, the nuclear area, cytoplasmic area, nucleus-to-cytoplasm ratio, and nuclear roundness factor were significantly higher in patients without aphthae. The neutrophil infiltration rate did not differ significantly in patients with or without aphthae. CONCLUSION: Behçet's disease can produce cytomorphometric changes in buccal cells that are detectable by exfoliative cytology and cytomorphometric analysis techniques.

A comparison of a 5% potassium hydroxide solution with a 5-fluorouracil and salicylic acid combination in the treatment of patients with anogenital warts: a randomized, open-label clinical trial.

Anogenital warts are caused by human papillomavirus (HPV), over 30 types of which are infectious for the anogenital tract. Without treatment, warts may regress spontaneously, remain unchanged, or increase in number and size. This study compared the efficacy of a topical 5% potassium hydroxide (KOH) solution with that of a topical 0.5% 5-fluorouracil (5-FU) and 10% salicylic acid (SA) combination in the treatment of anogenital warts. Sixty patients were randomly assigned to receive topical KOH or 5-FU + SA. Both groups demonstrated a significant decrease in numbers of lesions (P < 0.05), but this difference was not significant at week 12 (P > 0.05). The mean number of lesions decreased from baseline to week 12 from 17.03 ± 12.64 to 3.73 ± 7.30 and from 16.13 ± 12.97 to 3.10 ± 4.90 in the KOH and 5-FU + SA groups, respectively (P < 0.001). Excellent clearance was achieved by 70.0 and 76.7% of patients in the KOH and 5-FU + SA groups, respectively. Marked improvement was seen in 13.3 and 20.0% of patients in the KOH and 5-FU + SA groups, respectively. At week 16, relapse was observed in two patients in the KOH group and three in the 5-FU + SA group (P > 0.05). No serious adverse events were reported. Neither treatment was more efficacious. Safety and ease of application are important goals in treatments for anogenital warts. A 5% KOH solution is a promising alternative treatment because it is effective and inexpensive and causes minimal side effects.

Autologous Serum Skin Test versus Autologous Plasma Skin Test in Patients with Chronic Spontaneous Urticaria.

Previous studies indicate that 25-45% of chronic urticaria patients have an autoimmune etiology. Autologous serum skin test (ASST) and autologous plasma skin test (APST) are simple tests for diagnosing chronic autoimmune urticaria (CAU). However, there are still some questions about the specificity of these tests. This study consisted of 50 patients with chronic spontaneous urticaria (CSU) and 50 sex- and age-matched healthy individuals aged 18 years, and older. A total of 31 (62%) patients and 5 (10%) control patients had positive ASST; 21 (42%) patients and 3 (6%) control patients had positive APST. Statistically significant differences were noted in ASST and APST positivity between the patient and control groups (ASST P < 0.001; APST P < 0.001). Thirteen (26%) patients and 5 (10%) control patients had antithyroglobulin antibodies or antithyroid peroxidase antibody positivity. No statistically significant differences were noted in thyroid autoantibodies between the patient and control groups (anti-TG P = 0.317; anti-TPO P = 0.269). We consider that the ASST and APST can both be used as in vivo tests for the assessment of autoimmunity in the etiology of CSU and that thyroid autoantibodies should be checked even when thyroid function tests reveal normal results in patients with CSU.

Assessment of QRS duration and presence of fragmented QRS in patients with Behçet's disease.

BACKGROUND: QRS prolongation and the presence of QRS fragmentation in 12-lead ECG are associated with increased mortality and sudden cardiac death in the long term. In this study we aimed to assess QRS duration and fragmentation in patients with Behçet's disease (BD). METHODS: A total of 50 patients (mean age 42.7±12.0 years) previously diagnosed with BD were recruited. In addition, a control group consisting of 50 healthy people (mean age 39.4±12.5 years) was formed. The longest QRS duration was measured in surface 12-lead ECG and QRS complexes were evaluated in terms of fragmentation. Serum C-reactive protein levels were also obtained. RESULTS: QRS duration and corrected QT duration were significantly longer in patients with BD compared with controls (102.75±11.91 vs. 96.99±10.91 ms, P=0.007; 438.55±30.80 vs. 420.23±28.06 ms, P=0.003, respectively). Fragmented QRS (fQRS) pattern was more common in patients with BD than controls [n=27 (54%) vs. n=16 (32%), P=0.026]. Disease duration was longer in patients with BD with fQRS compared with those without (12.67±8.68 vs. 7.09±7.06 years, P=0.010). Furthermore, C-reactive protein level was higher in patients with BD with fQRS compared with those without (6.53±4.11 vs. 4.97±6.32 mg/dl, P=0.043). Correlation analysis revealed no association between disease duration and QRS duration (r=0.219, P=0.126). CONCLUSION: QRS duration is greater and fQRS complexes are more frequent in patients with BD. These findings may indicate subclinical cardiac involvement in BD. Given the prognostic significance of ECG parameters, it is reasonable to evaluate patients with BD with prolonged and fQRS complexes more in detail such as late potentials in signal averaged ECG in terms of cardiac involvement.

Serum prolidase activity in psoriasis patients.

This study aimed to evaluate serum prolidase activity and the effects of gender, body mass index (BMI), disease severity and duration, and therapy type on prolidase activity in patients with psoriatic as well as the relationship between serum NO· and prolidase levels in these patients. The study included 29 clinically documented plaque patients with psoriasis and 24 healthy volunteers. Data such as age, sex, BMI, duration and severity of disease, and type of therapy were assessed. NO· levels were determined by the Griess reaction. Serum prolidase assay is based on a colorimetric determination of proline by Chinard's reagent. We did not determine any difference in serum NO· levels of psoriatic patients when compared to controls. Serum prolidase levels in psoriasis patients were significantly higher than those in controls. There was no significant difference in prolidase activity between male and female. No statistically significant correlations were found between serum prolidase levels and BMI, PASI and disease duration. When compared between topical treatment group and systemic treatment group, there was no significant difference in serum prolidase activity. In conclusion, patients with psoriasis exhibit higher serum prolidase activity independent of gender, BMI, disease severity or duration, type of treatments or NO· level. However, further studies are needed to verify these findings as well as altered collagen synthesis in patients with psoriasis.

Investigation of the atrial electromechanical delay duration in Behcet patients by tissue Doppler echocardiography.

AIMS: To investigate the atrial electromechanical delay (EMD) duration that is a non-invasive predictor of atrial fibrillation (AF) in patients with Behcet's disease (BD). METHODS AND RESULTS: Thirty-eight Behcet's patients (24 females, 14 males; mean age: 43.6 ± 10.3 years) who were being followed in the dermatology or internal medicine department and 29 demographically matched controls (13 females, 16 males; mean: age 42.6 ± 11.1 years) were included in the study. The inclusion criteria were recurrent oral ulcerations and two of the following features: recurrent genital ulceration, eye lesions, skin lesions or positive pathergy skin test for Behcet's group. Using tissue Doppler imaging, atrial electromechanical coupling [time interval from the onset of P wave on surface electrocardiogram to the beginning of A wave interval with tissue Doppler echocardiography (PA)] were measured from the lateral mitral annulus (PA lateral), septal mitral annulus (PA septum), and right ventricular tricuspid annulus (PA tricuspid). The mean disease duration was 10.5 ± 7.7 years. The inter-atrial and intra-atrial EMD were significantly higher in the Behcet group than those in the controls (19.8 ± 8.2 vs. 13.1 ± 4.4 ms, P = 0.001; 11.5 ± 7.4 vs. 6.9 ± 3.7 ms, P = 0.02; respectively). The left atrial EMD was similar in both of the groups. However, the P(max) and PWD values were significantly higher in the BD group compared with those in the controls (120.5 ± 10.1 vs. 112.1 ± 5.9 ms, P < 0.0001; 44.9 ± 10.7 vs. 28.4 ± 5.9 ms, P < 0.0001; respectively). CONCLUSION: Atrial electromechanical conduction times were increased in the BD patients compared with those in the controls. The tendency of BD patients to go into AF can be easily and non-invasively detected using tissue Doppler echocardiography. These findings may be indicators for subclinical cardiac involvement.

Benign cephalic histiocytosis: a case report.

Histiocytic skin disorders are usually classified as either Langerhans' cell histiocytosis (LCH) or non LCH, based on the pathology. Benign cephalic histiocytosis (BCH) is a rare type of non-Langerhans histiocytitic disorder and is characterized by self-healing multiple small eruptions of yellow to red-brown papules on the face and upper trunk. Histologic features of this disorder show dermal proliferation of histiocytes that have intracytoplasmic comma-shaped bodies, coated vesicles and desmosome-like structures. In this study, we report on a 7-month-old boy who contained small yellow-red papules on his face that spread to his upper trunk. The clinical and histologic features in this patient were consistent with BCH.

Digital block with and without epinephrine during chemical matricectomy with phenol.

BACKGROUND: Digital block with epinephrine is safe in selected patients. Chemical matricectomy with phenol is a successful, cheap, and easy method for the treatment of ingrown nails. OBJECTIVE: To determine the effect of digital block with epinephrine in chemical matricectomy with phenol. MATERIAL AND METHODS: Forty-four patients with ingrown toenail were randomly divided into two groups. The plain lidocaine group (n=22) underwent digital anesthesia using 2% plain lidocaine, and the lidocaine with epinephrine group (n=22) underwent digital anesthesia with 2% lidocaine with 1:100,000 epinephrine. In the postoperative period, the patients were evaluated for pain, drainage, and peripheral tissue destruction and were followed for up to 18 months for recurrence. RESULTS: The mean anesthetic volume used in the epinephrine group (2.2 ± 0.4 mL) was significantly lower than the plain lidocaine group (3.1 ± 0.6 mL). There was no statistically significant difference in postoperative pain and recurrence rates, but duration of drainage was significantly shorter in the epinephrine group (11.1 ± 2.5 days) than in the plain lidocaine group (19.0 ± 3.8 days). CONCLUSION Digital block with epinephrine is safe in selected patients, and epinephrine helps to shorten the postoperative drainage period. The authors have indicated no significant interest with commercial supporters.

[Mediterranean spotted fever due to contact with dog-tick]. / Köpek kenesi ile temas sonrasinda gelisen akdeniz benekli atesi.

Mediterranean spotted fever (MSF) is one of the tick-borne rickettsial infections caused by Rickettsia conorii. It is transmitted to humans by brown dog ticks (Rhipicephalus sanguineus). In this case report, a 16-years-old male patient who was diagnosed as MSF after an exposure to dog-tick in Bartin province (located at middle Black Sea region of Turkey) has been presented. His history revealed that, five days before admission to the hospital (on June, 2007) he had cleaned dog-ticks from his dog, and after 12 hours he found a stucked tick on his leg and he took it out right away with a tweezer. High fever, headache and generalized maculopapular rash including soles and palms and a black-colored lesion at the tick bite site developed three days later. In clinical examination, there was a black escar circled with a red-purple colored halo in front of the right tibia at the site of the tick bite showing high similarity to "tache noire" which was specific to MSF. Indirect immunofluorescence assay (IFA) for Rickettsia yielded negative result in the serum sample collected on admission day, however, it was found positive at 1/512 titer in the serum sample collected 10 days after admission. The patient has recovered completely without any complication after 10 days of doxycycline therapy. The aim of this presentation is to point out that MSF should be considered for the differential diagnosis of a patient with a history of tick bite, fever, maculopapular rash, headache, myalgia, arthralgia and especially with black escar during summer months in our country where the incidence of tick-borne infections has been increasing since recent years.

Peripheral nervous system involvement in patients with Behçet disease.

BACKGROUND: Central nervous system involvement in Behçet disease (BD) has been well documented, but studies evaluating peripheral nervous system involvement are relatively uncommon. OBJECTIVE: The aim of this study is to evaluate the frequency and characteristics of peripheral nervous system involvement in BD. METHODS: Sixty-nine BD patients (36 women, 33 men) followed by neurology and dermatology outpatient clinics between October 1999 and October 2004 were enrolled into study. A careful history was taken and physical and neurologic examinations were done. All other medical causes that may be related to peripheral nervous system involvement were studied. All patients underwent nerve conduction studies using standard electrophysiologic procedures. RESULTS: Peripheral nervous system involvement was detected in 13 patients. There was no sign and symptom related to peripheral nervous system involvement in these patients. Causes other than BD were found in 5 patients with polyneuropathy. Asymptomatic delay in conduction velocities of the median nerves was detected in 3 patients. In 5 patients with clinically evident peripheral nerve involvement due to BD, 3 had sensorimotor and 2 had sensory axonal neuropathies. CONCLUSION: Axonal type polyneuropathy may be seen in BD patients. Peripheral involvement may be detected by electrophysiological examinations in asymptomatic BD patients.

Psychopathology, depression and dissociative experiences in patients with lichen simplex chronicus.

OBJECTIVE: Psychogenic factors may play a contributory role in the development or persistence of lichen simplex chronicus (LSC). The objective of this study was to evaluate the psychiatric profile of patients with LSC including depression and dissociative experiences. METHOD: Dermatology outpatients with a LSC (n=30) were compared with outpatients with tinea in which psychological factors are regarded as negligible (n=30). All subjects were given psychiatric scales including the Symptom Checklist-90-Revised (SCL-90-R), Hamilton Rating Scale for Depression (HAM-D) and Dissociative Experience Scale (DES). RESULTS: All mean SCL-90 scores for general psychopathology were higher in the LSC compared to the control group. HAM-D and DES scores were significantly higher in the LSC group (P<.05) as well. In addition, the number of patients whose total DES score of 30 and above was higher in the LSC group. CONCLUSIONS: Psychiatric symptoms appear relatively common among patients with LSC. Further research is needed to confirm the possible role of dissociative tendencies in the etiology of LSC.

Sleep disturbance in patients with lichen simplex chronicus and its relationship to nocturnal scratching: A case control study.

BACKGROUND: Lichen simplex chronicus (LSC) is a common pruritic disorder resulting from repeated rubbing and scratching. Nighttime pruritus is a common feature in LSC and may disrupt the sleep pattern. The aim of this study is to determine whether there are sleep abnormalities in patients with LSC. PATIENTS AND METHODS: Fifteen patients with LSC and 15 age-, sex- and body mass index-matched control subjects were enrolled in the study. No participant had any other medical or psychiatric illness. All subjects were evaluated by overnight polysomnography, scratch electrodes, Epworth sleepiness scale and a general questionnaire for demographic data and sleep problems. RESULTS: There were no significant differences in the groups for total sleep time, sleep efficiency, sleep latency, rapid eye movement (REM) latency, percentage of stage 1 non-REM sleep and REM sleep. The percentage of stage 2 non-REM sleep was higher (P < 0.05) and the percentage of slow wave sleep (stages 3 and 4) was lower in the study group (P < 0.05) than in the controls. The patient group had a mean of 15.9 +/- 7.5 arousal index and 22.8 +/- 14.1 awakenings compared with 9.5 +/- 3.1 and 10.4 +/- 3.9, respectively, in the controls (P < 0.05, P < 0.01, respectively). Arousals of patients were mainly observed in non-REM sleep. The number of scratching bouts ranged from 6 to 20 per night. Scratching episodes were observed frequently during stage 2 non-REM sleep. CONCLUSION: Polysomnographic findings of patients with LSC demonstrated that sleep structure is disturbed by arousals and awakenings related to scratching bouts during sleep.