Assessment of dynamic thiol-disulfide homeostasis in patients with lipoid proteinosis (Urbach-Wiethe syndrome).

OBJECTIVE: Lipoid proteinosis is a rare autosomal recessive genetic dermatological disease that occurs due to the accumulation of hyaline material in the skin and mucous membranes. This study aimed to investigate whether dynamic thiol-disulfide homeostasis is a new marker of oxidative stress in patients suffering from lipoid proteinosis. METHODS: The study group involved 17 patients with lipoid proteinosis and 17 healthy controls with same gender and age. Native thiol, total thiol, disulfide levels, and thiol-disulfide indexes were measured with the fully automated spectrophotometric method described by Erel and Neselioglu, and the results of the two groups were statistically analyzed. RESULTS: Serum total thiol and native thiol levels were significantly lower in lipoid proteinosis group compared to the control group (p=0.020 and p=0.014, respectively). The disulfide levels were found to be higher in lipoid proteinosis group, but there was no significant difference between two groups. CONCLUSIONS: Impaired dynamic thiol-disulfide homeostasis was observed in lipoid proteinosis patients, suggesting that thiol-disulfide homeostasis may have a role in the pathogenesis of this disease.

Assessment of dynamic thiol-disulfide homeostasis in patients with lipoid proteinosis (Urbach-Wiethe syndrome)

SUMMARY OBJECTIVE: Lipoid proteinosis is a rare autosomal recessive genetic dermatological disease that occurs due to the accumulation of hyaline material in the skin and mucous membranes. This study aimed to investigate whether dynamic thiol-disulfide homeostasis is a new marker of oxidative stress in patients suffering from lipoid proteinosis. METHODS: The study group involved 17 patients with lipoid proteinosis and 17 healthy controls with same gender and age. Native thiol, total thiol, disulfide levels, and thiol-disulfide indexes were measured with the fully automated spectrophotometric method described by Erel and Neselioglu, and the results of the two groups were statistically analyzed. RESULTS: Serum total thiol and native thiol levels were significantly lower in lipoid proteinosis group compared to the control group (p=0.020 and p=0.014, respectively). The disulfide levels were found to be higher in lipoid proteinosis group, but there was no significant difference between two groups. CONCLUSIONS: Impaired dynamic thiol-disulfide homeostasis was observed in lipoid proteinosis patients, suggesting that thiol-disulfide homeostasis may have a role in the pathogenesis of this disease.

Cutaneous Leishmaniasis due to Three Leishmania Species Among Syrian Refugees in Sanliurfa, Southeastern Turkey.

Sanliurfa, a city in southeastern Turkey, is host to 477,166 Syrian refugees. The incidence of cutaneous leishmaniasis (CL) may be on the rise in areas affected by a refugee crisis, like Sanliurfa; thus, consequently, local uncommon species of Leishmania may be encountered in these regions. This might potentially make diagnosis and treatment more challenging over time. In this study, it was aimed to identify the causative agents of CL in clinical samples. A total of 154 patients (60 Syrian and 94 Turkish) who were diagnosed with CL via microscopical examination and PCR were enrolled this study. All of the samples were analyzed using internal transcribed spacer 1 genes, restriction fragment length polymorphism, DNA-sequencing, and phylogenetic analyses. In this study, Leishmania tropica was determined to be the predominant species in 140 of the patients (90.9%), followed by Leishmania major in 12 patients (7.8%), and Leishmania infantum in 2 patients (1.3%). Of the 94 Turkish patients, 94.7% were infected with L. tropica and 5.3% were infected with L. major, while none were infected with L. infantum. However, of the 60 Syrian patients, 85% were infected with L. tropica, 11.7% were infected with L. major, and 3.3% were infected with L. infantum. There was a significant association between the Leishmania species and the nations (Turkish-Syrian) (P < 0.001). The sequences were numbered from MH347941 to MH347953 and submitted to GenBank. This study confirmed that L. tropica, L. major, and L. infantum coexisted in Sanliurfa. This was the first time that the species L. infantum was reported among recent immigrants from Syria in Sanliurfa. Therefore, it is necessary to discriminate the Leishmania species for diagnosis, treatment, and controlled studies in hyper-endemic regions.

Clinical practice guidelines for the diagnosis and treatment of cutaneous leishmaniasis in Turkey.

BACKGROUND: Cutaneous leishmaniasis (CL) is a vector-born parasitic disease characterized by various skin lesions that cause disfiguration if healed spontaneously. Although CL has been endemic for many years in the southern regions of Turkey, an increasing incidence in nonendemic regions is being observed due to returning travelers and, more recently, due to Syrian refugees. Thus far, a limited number of national guidelines have been proposed, but no common Turkish consensus has emerged. OBJECTIVES: The aim of this study was to develop diagnostic and therapeutic guidelines for the management of CL in Turkey. METHODS: This guideline is a consensus text prepared by 18 experienced CL specialists who have been working for many years in areas where the disease is endemic. The Delphi method was used to determine expert group consensus. Initially, a comprehensive list of items about CL was identified, and consensus was built from feedback provided by expert participants from the preceding rounds. RESULTS: Evidence-based and expert-based recommendations through diagnostic and therapeutic algorithms according to local availability and conditions are outlined. CONCLUSION: Because CL can mimic many other skin diseases, early diagnosis and early treatment are very important to prevent complications and spread of the disease. The fastest and easiest diagnostic method is the leishmanial smear. The most common treatment is the use of local or systemic pentavalent antimony compounds.

A Case Of Bilateral Acquired Localized Lipoatrophy.

Lipoatrophy is characterized by inflammation and tissue loss in fatty tissue. This disease may be congenital or acquired, primary or secondary. Secondary lipoatrophy develops with infections, collagen tissue diseases, tumors and drug injections. In this report, we present the case of a 14-year-old female patient who developed lipoatrophy following intramuscular steroid injection to both buttocks.

Pediatric Cutaneous Leishmaniasis in an Endemic Region in Turkey: A Retrospective Analysis of 8786 Cases during 1998-2014.

BACKGROUND: Cutaneous leishmaniasis (CL) is a major public health concern in Turkey and Sanliurfa represents the most endemic city in Turkey. Although children are most commonly affected by CL, detailed studies of pediatric CL in Turkey are lacking. METHODOLOGY/PRINCIPAL FINDINGS: In this report we retrospectively evaluated clinical and epidemiological data of 8786 pediatric CL cases, and how children respond to antimonial therapy. CL was observed most frequently in children between 6-10 years old. Interestingly this group showed shorter duration of disease and smaller lesions compared to 0-5 year and 11-15 year old groups. Females were more affected in all groups. Lesion localization and types varied among groups, with 0-5 year old presenting head/neck and mucosal lesions, and more often suffered from recidivans type, this could be associated to the longest duration of the disease in this group. Eleven-15 year old group showed fewer lesions in the head/neck but more generalized lesions. Evaluation of treatment response revealed that intra-lesional treatment was preferred over intramuscular treatment. However, 0-5 year old received intramuscular treatment more often than the other groups. Furthermore, the majority of 0-5 year old group which received intra-lesional treatment did not received subsequent intra-lesional cycles, as did children in the range of 6-15 years old. CONCLUSIONS/SIGNIFICANCE: We report an increase in pediatric CL patients within the last four years. Analysis of pediatric CL patients by age revealed significant differences in CL progression. The data suggest that children between 0-5 years old responded better than other groups to intralesional treatment, since they received more often a single cycle of IL treatment, although follow up observation is required since they were more prone to develop recidivans. Eleven-15 year old patients comprise the largest percentage of patients receiving two or three cycles of intralesional treatment, suggesting that this group did not respond efficiently to intralesional treatment and highlighting the need for more effective therapeutic strategies against CL.

[Investigation of cutaneous leishmaniasis by active screening in primary schools in Sanliurfa, Turkey]. / Sanliurfa'da ilkögretim okullarinda aktif tarama yöntemiyle kütanöz leysmanyaz arastirmasi.

Cutaneous leishmaniasis (CL) is endemic in Sanliurfa, a province located at southestern part of Turkey. The aims of this study were to determine CL cases among children aged 7-15 years attending primary schools and evaluate the demographical and clinical characteristics by active screening method. In our study, a total of 163.464 children attending to 123 primary schools have been evaluated in terms of CL prevalence between 2010 to 2012 period. Accordingly, 67.585, 72.279 and 23.600 students studying in 74, 37 and 12 primary schools were actively screened, respectively. Children suspected with CL were invited to the Center for Diagnosis and Treatment of Oriental Sore, Sanliurfa Division of Turkish Public Health Institute. Two slides were prepared from bloodless, serous fluid of the skin lesions and stained with Giemsa for the microscopic examination to identify Leishmania amastigotes. The lesions were also evaluated according to their clinical manifestations, size, duration and number. In the study, 455 (%0.3) of the children were diagnosed as CL. The incidence of CL cases was 0.41%, 0.14%, and 0.31% in 2010, 2011 and 2012, respectively. Of the CL cases, 250 (54.9%) were female and 205 (45.1%) were male with a mean age of 10.54 ± 2.42 years. Most of the cases (64.2%) were in 7-11 years old group, while 35.8% were in 12-15 years old group. The average number of the CL lesions was 1.78±0.7, ranging from one to fifteen. Single lesions were detected in 281 cases (61.8%), whereas double, triple and multiple lesions (≥ 4) were detected in 87 (19.1%), 51 (11.2%) and 36 (7.9%) of the CL cases, respectively. The lesions were mostly involved on face (n= 280, 61.5%) and especially cheeks (133/280, 47.5%), followed by upper (n= 127, 27.9%) and lower (n=39, 8.6%) extremities. The average duration of lesions was 13.40 ± 0.47 months (range: 4-104 weeks) and the average size of lesions was 10.32 ± 0.4 mm (range: 3-10 mm). Among all, mostly nodular (n= 211, 46.4%) and ulcerative (n=204, 44.8%) lesions were recorded. Of the cases 330 (72.5%) were diagnosed as acute CL (duration of lesions were ≤ 24 months), while 125 (27.5%) were chronic CL (duration of lesions were > 24 months). Leishmaniasis recidivans were identified in a total of five (1.1%) cases, of them one was acute and four was chronic CL cases. Twenty-five relatives of the children with CL were diagnosed as new CL cases in the 2011-2012 period. Most of the patients (n= 437, 96%) were successfully treated with topical pentavalent antimonials, however 18 (4%) who were unresponsive to local therapy were treated systemically. Our findings showed that local transmission is ongoing in terms of CL. It should be kept in mind that, primary school aged children might be responsible for the ongoing local transmission in endemic regions and the local transmission may be decreased with the early diagnosis and treatment of CL cases.

Meglumine antimoniate is more effective than sodium stibogluconate in the treatment of cutaneous leishmaniasis.

Sodium stibogluconate (SSG, Pentostam) and meglumine antimoniate (MA, Glucantime) are two antimonials that are widely used to treat cutaneous leishmaniasis (CL), but the relative efficacies of these treatments are not clear. The aim of this study is to compare the efficacy of intralesional SSG with intralesional MA therapy in the treatment of CL. One month after completion of the therapy, 1431 of 1728 patients (82%) who received intralesional MA showed complete clinical cure compared to 1157 of 1728 patients (67%) in the SSG group. Patients who did not respond to the first round of therapy were re-administered the same treatment but with twice weekly injections. Following completion of the second course of therapy, 237 of 297 patients (80%) in the MA group and 407 of 561 patients (72%) in the SSG group healed their lesions by 1-month post-treatment. At both times, the differences in cure rates between MA and SSG groups were statistically significant (p < 0.05). Cure rates in the MA group were always significantly higher than SSG groups irrespective of other parameters including age, gender, lesion site and type of lesion. Intralesional MA is more effective than intralesional SSG in the treatment of CL.

Juvenile dermatomyositis with joint contractures and calcinosis cutis.

A 12-year-old boy presented to our department with firm papules on the fingers of both hands, erythematous scaly plaques on the dorsum of the hands and elbow, and deformities and limitation of motion in the joints of the hands and feet. His parents reported that the eruption started 6 years prior to presentation. He was previously diagnosed with psoriasis by physicians and acitretin treatment was given. However, he did not benefit from the treatment.

A Comparison of Demographic and Clinical Characteristics of Syrian and Turkish Patients with Cutaneous Leishmaniasis.

Cutaneous leishmaniasis (CL) is a significant public health problem with increasing incidence, especially in extreme circumstances. In this study, we compared the sociodemographic and clinical characteristics of 685 Syrian CL patients afflicted by the Syrian conflict and 685 Turkish CL patients in 2012. Patient age, gender, duration of disease, lesion size, type, and localization were evaluated. The duration of CL disease in Syrian CL patients (4.5 ± 4.3 weeks) was shorter than that of Turkish CL patients (11.9 ± 9.7 weeks). The number of lesions was greater in Syrian patients (2.46 ± 2.43) than in Turkish patients (1.93 ± 1.47). Lesion sizes were comparable between both groups (Syrian, 11.2 ± 8.7 mm; Turkish, 10.7 ± 7.7 mm). In Syrian CL patients, nodular type lesions were the most common (325 patients, 49.1%), whereas, in Turkish CL patients, ulcer type lesions were the most common (352 patients, 51.5%). Our results indicate variations in the clinicoepidemiological features of CL between Turkish and Syrian patients within Sanliurfa province. This highlights the impact of social unrest and environmental conditions on the epidemiology of CL within this region. Approaches to prevention, control, and treatment of CL in these areas should take into consideration the emerging changes in clinicoepidemiological parameters of the disease.

Prolidase activity in chronic plaque psoriasis patients.

INTRODUCTION: Psoriasis is a chronic, inflammatory, T-cell-mediated and hyperproliferative skin disease characterized by erythematous, squamous, sharply circumscribed and infiltrated plaques. The metabolisms of the collagen proteins undergo considerable changes due to the acceleration of their turnovers as a result of increased prolidase activity in psoriasis patients. AIM: To determine the level of prolidase activity in psoriasis patients and evaluate its relationship with the oxidative system. MATERIAL AND METHODS: The serum prolidase enzyme activity, total antioxidant levels and total oxidant levels of 40 psoriasis patients and a control group including 47 healthy individuals were analyzed by using their serum samples, and their oxidative stress indices were calculated. RESULTS: The prolidase levels (p < 0.01), total oxidant levels (p < 0.01) and oxidative stress index levels (p < 0.001) of the patient group were higher than the corresponding parameters in the control group. The total antioxidant level was low (p < 0.01). Although a positive correlation was found between the prolidase and total antioxidant levels and the total oxidant level, no correlation was found between prolidase and the oxidative stress index. CONCLUSIONS: It has been determined that the activity of the prolidase enzyme increases due to the increased collage turnover in psoriasis patients. Increased serum oxidant levels and oxidative stress indices values may play a role in the pathogenesis of psoriasis.

[Successful treatment of cutaneous leishmaniasis with amphotericin B; a case of unresponsive to pentavalent antimony therapy]. / Sistemik Lipozomal Amfoterisin B Tedavisine Cevap Veren Kutanöz Leishmaniasis Olgusu.

Cutaneous Leishmaniasis (CL) is a skin infection caused by various species of Leishmania parasites, which is transmitted by infected Phlebotomus sandfly bites. Pentavalent antimonials (meglumine antimoniate and sodium stibogluconate) are used for the treatment of adult CL patients as an effective and safe method. Liposomal amphotericin B is an alternative for the treatment of choice in cutaneous leishmaniasis cases which pentavalan antimony contraindicated or unresponsive to pentavalent antimony therapy. In this study, successful treatment with systemic liposomal amphotericin B of a cutaneous leishmaniasis case developing local side effects related both systemic and intralesional meglumine antimonate treatment was presented.

Assessment of psychiatric morbidity and quality of life in children and adolescents with cutaneous leishmaniasis and their parents.

INTRODUCTION: Cutaneous leishmaniasis (CL) is a common parasitic infectious disease caused by different species of the leishmania genus. The skin lesions are usually found on exposed areas, especially the face, arms and legs. Although the disease does not cause significant morbidity, the lesions can be troublesome and unsightly. The disease have negative impacts in areas such as patients' psychological well-being, social life and daily activities in adults. AIM: To determine the frequency of psychiatric morbidity in children and adolescents who have cutaneous leishmaniasis (CL) and to determine the effect of CL on their levels of depression and anxiety and on their quality of life (QoL). MATERIAL AND METHODS: Fifty-four patients with CL (29 males and 25 females), who were 7 to 18 years of age, were assessed with the Child Depression Inventory (CDI) and the State-Trait Anxiety Inventories for Children (STAIC). The patients and their mothers were assessed with the Pediatric Quality of Life Inventory Parent and Child Versions (PedQL-P and C, respectively). This questionnaires were filled in by the control group consisting of 40 healthy children and adolescents (20 males and 20 females) and their parents from the local community matched for age, gender, and education level of the parents. RESULTS: Both the patient group and the control group had high scores on the depression measurement scale (t = 5.36, p < 0.05). These measurements also show significant differences between children and adolescents, who were defined as 12 years of age and under as well as older than 12 years, respectively (12 years of age and under (t = 3.14, p = 0.04); over 12 years (t = 5.37, p < 0.001)). However, there was no significant difference between the anxiety scores of the general patient group and the control group when classified according to age. The anxiety sensitivity index scores did not differ in either group from those of the control group. The patients' and the mothers' QoL scores for all of the scales, including all subscale scores, were significantly different from those of the control group (both 12 years of age and under as well as older than 12 years). CONCLUSIONS: The results have shown that the frequency of depressive symptoms is much higher in patients who have CL than in healthy controls. In addition, the QoL of children and adolescents with CL and of their mothers was found to be much lower than that of the control group. Therefore, the follow-up for patients with CL who are referred to dermatology clinics should include a psychiatric evaluation. If necessary, they should be referred for psychiatric support.

Ulcerative penile leishmaniasis in a child.

Penile ulcers may be caused by several different agents. Rarely, cutaneous leishmaniasis may also be accompanied by penile ulcers. We report a five-year-old boy with who had an ulcer on the glans penis. Smears from the ulcer demonstrated amastigotes, biopsy showed histopathological features of leishmaniasis and Leishmania was grown in culture. Treatment with meglumine antimoniate injections led to improvement.