Epidemiology of pemphigus in Turkey: One-year prospective study of 220 cases

Pemphigus is a group of rare and life-threatening autoimmune blistering diseases of the skin and mucous membranes. Although they occur worldwide, their incidence shows wide geographical variation, and prospective data on the epidemiology of pemphigus are very limited. Objective of this work is to evaluate the incidence and epidemiological and clinical features of patients with pemphigus in Turkey. All patients newly diagnosed with pemphigus between June 2013 and June 2014 were prospectively enrolled in 33 dermatology departments in 20 different provinces from all seven regions of Turkey. Disease parameters including demography and clinical findings were recorded. A total of 220 patients were diagnosed with pemphigus during the 1-year period, with an annual incidence of 4.7 per million people in Turkey. Patients were predominantly women, with a male to female ratio of 1:1.41. The mean age at onset was 48.9 years. Pemphigus vulgaris (PV) was the commonest clinical subtype (n=192; 87.3%), followed by pemphigus foliaceus (n=21; 9.6%). The most common clinical subtype of PV was the mucocutaneous type (n=83; 43.2%). The mean Pemphigus Disease Area Index was 28.14±22.21 (mean ± Standard Deviation).  The incidence rate of pemphigus in Turkey is similar to the countries of South-East Europe, higher than those reported for the Central and Northern European countries and lower than the countries around the Mediterranean Sea and Iran. Pemphigus is more frequent in middle-aged people and is more common in women. The most frequent subtype was PV, with a 9-fold higher incidence than pemphigus foliaceus.   </p

Investigation of Frequency of Pemphigus Autoantibodies in Healthy First Degree Relatives of Pemphigus Patients

WOS: 000277987300018Objective: Many observation and investigations suggests that inheritance plays a role in the pathogenesis of pemphigus, which is an autoimmune disease. Many familial cases have been reported up to date. The purpose of our study is to evaluate the autoantibody levels that deposit in the tissue and circulate in the serum, in unaffected first degree relatives of pemphigus patients in our region by using indirect immunufluorescence test and ELISA. We also aim to detect the presence and frequency of specific antibody levels. Material and Methods: Sixty eight first degree relatives of 29 pemphigus patients, who had neither pemphigus nor another chronic autoimmune disease were included in our study. Indirect immunufluorescence test was done to detect autoantibodies in serum of the relatives and controls by using monkey and rat esophagus as a. ELISA was done in 32 relatives. Direct immunofluorescence examination was done in 13 relatives whose serum titration was positive when rat esophagus was used as a substrate. Results: Positive results were obtained in 28% of relatives (between 1/10-1/80) and 16% of control groups when rat esophagus was used as a substrate and 10% of relatives (1/10) and 8% of control groups when monkey esophagus used as a substrate. None of the 32 relatives, whose serum examined by ELISA, had antibodies against the anti-desmoglein-1 whereas serum antibody against anti-desmoglein-3 was detected only in one. Direct immunufluorescence test was negative in all 13 relatives. Conclusion: Existence of circulating antibody levels was observed only in the presence of some substrates and at certain titrations in first degree relatives of our patients when compared to the healthy control group. According to our findings, we may suggest that only existence of circulating antibodies is not enough for the disease to appear, there may also be a barrier for these antibodies to precipitate into the skin

Severe inflammatory and keloidal, allergic reaction due to para-phenylenediamine in temporary tattoos

Hair coloring with henna has been popular in Turkey for years. In recent years since the tattoos are applied by the street vendors in most of the beach places in Turkey, skin coloring with henna has also increased. Henna can be used alone or in combination with other coloring agents. Henna alone can be safe but due to additives such as para-phenylenediamine (PPD), p-toluenediamine and various essential oils, allergic contact reactions may occur. We report a 22-year-old man who developed severe inflammatory and keloidal, moderately bullous allergic reaction after henna paint-on tattoo. We did a patch test separately with these painting products (henna powder, PPD) and with the European standard series. PPD was strongly positive (+++) on day 2 and remained positive for following days. After treating with topical clobetasol-17 butyrate, resolution was obtained in two weeks. But some keloidal reaction remained

The influence of training on the recognition of gross features of dermoscopy images

Background: In a dermoscopic examination, besides structural components, inexperienced clinicians should also be able to recognize the gross features of the images. Aim: The aim of this study is, whether or not an inexperienced clinician has problems in the recognition of gross features of the images on dermoscopic examination. Methods: Two dermatologists, of whom one was experienced in the field of dermoscopy and the other was not, examined 161 dermoscopic images of melanocytic lesions in the gross features of their borders. Inner and outer borders were defined for each lesion. Both dermatologists separately evaluated the borders of the lesions for irregularity, asymmetry, and wideness of fading. For subjective image analysis they scored each lesion by using the four-point ordinal scale. For computerized image analysis they manually marked borders with dots, by using a computer program. We used quadratic-weighted kappa for interobserver reliability assessments for subjective scores and intraclass correlation coefficients (ICC) for automatically calculated scores. Results: In a subjective evaluation the inexperienced observer used a higher score than the experienced observer and the kappa values were between 0.241– 0.286. ICC for the automatically calculated scores were between 0.357 and 0.522. According to both the outer and the inner borders, the concordance between experienced and inexperienced observers was almost perfect in measurements of diameter, perimeter, and area (ICC scores were between 0.948 and 0.990). Conclusions: An inexperienced person, in comparison with an experienced person, sees lesions in the same sizes, but in different shapes on dermoscopy. Therefore, it is advisable that making learners familiar with the borders of lesions should be included in the training on dermoscopy

Evaluation of a Guideline for Etiological Diagnosis of Chronic Urticaria

WOS: 000283186700022Background: Exhibiting potential causes of chronic urticaria in routine daily practice is a complex procedure. A guideline, developed by the researchers, investigating the etiology of chronic urticaria was evaluated in this study. In this guideline erythrocyte sedimentation rate, complete blood count, urine analysis and direct microscopic examination of stool for parasites were routine and other diagnostic procedures were requested according to clinical findings of potential causes. Objective: The purpose of the study was to determine the rate of implementation of the guideline considering a daily practice. Material and Methods: Following diagnosis of chronic urticaria, patients were interrogated with a standard questionnaire form, and were examined in order to determine the clinical findings of possible causes of the disease. The records of 903 patients in total were retrospectively evaluated. During the evaluation process, the necessary and unnecessary diagnostic procedures regarding the guideline were determined by using computerized algorithms. During evaluation some parameters such as age and gender of patients, duration and severity of the disease, and numbers and types of diagnostic procedures affecting the grade of the request and completion were also taken into account. Results: The total number of necessary procedures was 4070, less than one third of which were requested by the physicians. However, the patients completed more than three fourths of the requested procedures. Moreover, the physicians requested some unnecessary procedures in almost one third of the patients. Conclusion: According to clinical findings, lower threshold for necessity of investigation leads to more failure in implementation of a guideline. Guidelines developed for the etiological diagnosis of chronic urticaria should be evaluated retrospectively on data from daily practices to evaluate the utility of a guideline

Psoriasiform Drug Eruption Associated with Sodium Valproate

As psoriasis is a common skin disorder, knowledge of the factors that may induce, trigger, or exacerbate the disease is of primary importance in clinical practice. Drug intake is a major concern in this respect, as new drugs are constantly being added to the list of factors that may influence the course of the disease. We report a patient with a psoriasiform drug eruption associated with the use of sodium valproate. Physicians should be aware of this type of reaction. Early detection of these cases has practical importance since the identification and elimination of the causative drug are essential for therapy success

CTLA-4

WOS: 000406494200005Objective: Pemphigus, expressing a life-threatening blistering disease, result from autoantibodies against the proteins that mediate intercellular adhesion in desmosomes, namely desmoglein 1 and/or 3. The importance of cytotoxic T lymphocyte-associated antigen (CTLA)-4 in negative regulation of T lymphocytes, which take part in this autoimmune reaction, is well known. Gene polymorphisms regarding this molecule affect autoimmunity. We aimed to determine whether CTLA-4 +49 A/G, -318 C/T, -1661 A/G and CT60 A/G gene polymorphisms cause susceptibility to pemphigus in Turkish population. Methods: We detected genotypes of the single nucleotide polymorphisms for 118 pemphigus patients and 108 healthy individuals with the help of polymerase chain reaction-restriction fragment length polymorphism method. Results: Distribution of the CTLA-4 +49 A/G, -318 C/T, -1661 A/G and CT60 A/G allele and genotype frequencies did not differ between pemphigus patients and healthy controls (p=0.643, OR=0.931; p=0.847, OR=1.160; p=0.968, OR=0.975; p=0.173, OR=1.303, respectively). Conclusion: We concluded that these polymorphisms are not associated with pemphigus susceptibility in Turkish population. This is the first study investigating the possible role of the 4 CTLA single nucleotide polymorphism in pemphigus susceptibility simultaneously. The role of CTLA-4 -1661 A/G gene polymorphism in pemphigus was not studied previously

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

WOS: 000254315000003PubMed ID: 18045733Background: Behcet'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 131). Methods: Patients with initial symptoms at age 16 years or younger were considered as having juvenileonset 131). In all, 83 patients with juvenile-onset BID (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 131) and 31.66 +/- 8.71 years in adult-onset 131). 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 BID, except neurologic and gastrointestinal involvement, which were higher in juvenileonset 131) 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 131) were similar, except genital ulcer, which was higher in adult-onset 131) (P =.025). Limitations: Our study consisted of patients with 131) 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