Association of NFKB1 -94ATTG ins/del polymorphism (rs28362491) with pemphigus vulgaris.

Pemphigus vulgaris is a rare chronic blistering skin disease resulting from IgG autoantibodies directed against transmembrane desmosomal glycoprotein desmoglein 3 and is the most common form of pemphigus. Since interleukin-1 receptor-associated kinase (IRAK-1)/nuclear factor-kappa B (NF-kappa B) pathway plays an essential role in the pathogenesis of autoimmune diseases, the aim of the present study was to explore the role of polymorphisms in three genes, named IRAK1 (rs3027898), NFKBIA (rs696) and NFKB1 (-94ATTG insertion/deletion variant, - rs28362491), in PV susceptibility. Forty-four unrelated patients with PV (23 males) were enrolled in the study. Additionally, 77 ethnic matching healthy volunteers (45 males) with no personal or family history of chronic autoimmune or infectious diseases were studied. Strong statistical significant difference was observed between PV patients and controls for polymorphism -94 insertion/deletion ATTG in the promoter region of NFKB1 gene (P = 0.00005). Additional dedicated studies in larger groups of patients of various ethnicities are needed to replicate and confirm the preliminary findings.

Bullous pemphigoid in adolescence.

Bullous pemphigoid (BP) is the most common autoimmune blistering disease affecting the elderly but is quite rare in childhood. The majority of pediatric cases have been reported during early childhood. Adolescence is divided into three phases: early (10-13 years), middle (14-17), and late (18-21). This review aimed to identify BP cases in adolescence and demonstrate their clinical features and course. Our literature search was performed in Medline with the terms "bullous pemphigoid in childhood and adolescence," "childhood bullous pemphigoid," "juvenile bullous pemphigoid," and "autoimmune blistering and autoimmune bullous diseases in childhood." The data extraction for late adolescence was limited by the fact that this age group is included in adult BP registries. We identified nine cases in early adolescence. Mucosa were affected in 5 of 9 cases. Treatment consisted of systemic prednisone (8/9), in combination with dapsone (2/9), azathioprine (2/9), or erythromycin/nicotinamide (1/9). Relapses were reported in 3 of 9 cases. We identified five cases occuring in middle adolescence. Mucosa were not affected. Treatment consisted of systemic prednisone (5/5), in combination with dapsone (3/5), azathioprine (2/5), doxycycline/nicotinamide (1/5), or mycophenolate mofetil (1/5). Relapses were reported in two of five cases. No case of BP in the late adolescence was included in the results, as only one case met the search criteria, and overlapped with pemphigus vulgaris. With only 14 cases found in our review, BP in adolescence appears even rarer than in earlier childhood. Despite its low prevalence, BP should be included in the differential diagnosis of autoimmune blistering diseases in adolescents.

Serum Leptin, Resistin, and Adiponectin Concentrations in Psoriasis: A Meta-Analysis of Observational Studies.

BACKGROUND: Psoriasis is an immune-mediated, inflammatory disease. Adipokines contribute to the regulation of immune-mediated processes and inflammation. OBJECTIVE: The aim of our study was to systematically review the literature for studies that have evaluated the circulating concentrations of adipokines in patients with psoriasis and controls and to meta-analyze the best evidence available. METHODS: Eligible were studies that have assessed leptin, resistin, or adiponectin concentrations in psoriatic patients and a reference group. The study was conducted in adherence with the PRISMA standards. RESULTS: Psoriatic patients had higher leptin concentrations compared to controls (random effects model, mean difference, MD = 5.64 ng/mL, 95% CI: 3.00-8.29, p < 0.0001). Heterogeneity among studies was high (I2 = 95.9%). Psoriatic patients had higher resistin concentrations compared to controls (MD = 4.66 ng/mL, 95% CI: 2.62-6.69, p < 0.0001). Heterogeneity was high (I2 = 99.2%). Finally, psoriatic patients had lower adiponectin concentrations compared to controls (MD = -1.87 µg/mL, 95% CI: -2.76 to -0.98, p < 0.0001). Heterogeneity was high (I2 = 95.9%). CONCLUSION: The study supported the hypothesis that leptin and resistin concentrations are higher and adiponectin concentrations are lower in patients with psoriasis compared to controls. Hereby, the suggested pathogenic link between psoriasis and metabolic syndrome/obesity is reinforced and the role of comorbidities in psoriasis is highlighted.

Quality of Life in Greek Patients with Autoimmune Bullous Diseases Assessed with ABQOL and TABQOL Indexes.

is missing (Short communication).

Prevalence of onychomycosis among patients with nail psoriasis who are not receiving immunosuppressive agents: Results of a pilot study.

Nail psoriasis and onychomycosis can often be hard to differentiate clinically and may coexist, complicating each other's course. The aim of this study was to determine the prevalence of onychomycosis among patients with nail psoriasis not being treated with immunosuppressive agents, which constitute an independent risk factor for fungal infections. A cross-sectional study was performed. All adult patients with nail psoriasis who were not receiving antifungal and/or immunosuppressive treatment were recruited at the 2nd University Dermatology Department of Aristotle University of Thessaloniki from 10/2016 till 02/2017. If onychomycosis was clinically suspected, nail samples were collected and direct microscopy with 15% KOH solution and culture were performed. Target-NAPSI and DLQI score were also calculated. Of the 23 patients recruited, 20 were men and 3 were women, with a mean age of 53.43 years (48.25, 58.62), a mean target-NAPSI score of 10.72 (9.62, 11.77) and a mean DLQI score of 10.17 (7.46, 12.89). A total of 34.78% of patients tested positive for onychomycosis. Yeast were isolated in 37.50% of cases, non-dermatophyte filamentous fungi in 37.50% and T. rubrum in 12.50%. The prevalence of onychomycosis among nail psoriasis patients is higher than that among the general population of Greece (15%-20%). Yeast and moulds predominate in infection cases of nail psoriasis patients.

Clinical significance of anti-desmoglein-1 and -3 circulating autoantibodies in Pemphigus Patients Measured by Area Index and Intensity Score.

Detection of anti-desmoglein-1 (anti-DSG-1) and anti-DSG-3 autoantibodies is widely used in the diagnosis of pemphigus. Two validated scoring systems, Pemphigus Disease Area Index (PDAI) and Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), are used for the evaluation of clinical severity. The aim of this cross-sectional study was to interpret the titres of pemphigus autoantibodies in correlation with either total or location-dependent PDAI scores and ABSIS. A total of 35 pemphigus patients were selected and evaluated at 3 time points. Total PDAI and ABSIS seemed useful in pemphigus with cutaneous lesions or in the mucocutaneous form, while location-dependent PDAI and ABSIS scores were useful in the mucosal form. Anti-DSG-1 autoantibodies titres better showed the disease extent in pemphigus with cutaneous only or with mucocutaneous lesions. Anti-DSG-3 autoantibodies titres did not correlate to disease activity.

Benign cephalic histiocytosis: case report and review of the literature.

Benign cephalic histiocytosis (BCH) is a rare type of non-Langerhans histiocytic disorder, usually presenting with small, yellow-red or yellow-brown, asymptomatic papules, located mostly on the head and neck of infants and young children. The histopathologic hallmark of BCH is a well-circumscribed histiocytic infiltrate in the superficial to mid-reticular dermis. BCH is a self-healing disorder, with the eruptions regressing spontaneously in most cases; therefore no treatment is required. We present a case of BCH in a 7-month-old boy who was referred to our department with multiple, asymptomatic, yellow-red papules located on his face, and we review 55 cases published in the English-language literature in an effort to better describe and understand this unusual entity.

Serum levels of TNF- α , IL-12/23 p40, and IL-17 in psoriatic patients with and without nail psoriasis: a cross-sectional study.

Nail involvement has started playing a major role in the overall assessment and management of psoriatic disease. Biologics indicated for moderate to severe chronic plaque psoriasis are shown to be beneficial in nail disease. This study aimed to assess and compare the serum levels of TNF-α, IL-12/23 p40, and IL-17 in psoriatic patients with and without nail involvement. 52 consecutively selected patients with chronic plaque psoriasis were included in this cross-sectional study. Patients were studied and analyzed after they had been divided into 2 groups regarding the presence (n = 24) or not (n = 28) of nail psoriasis. The mean serum levels of TNF-α were significantly higher in the group of psoriatic patients with nail lesions compared to those without (t-test; 5.40 ± 1.17 versus 3.80 ± 1.63, P = 0.026). However, the median serum levels of both IL-12/23 p40 (Mann-Whitney; 92.52 (34.35-126.87) versus 150.68 (35.18-185.86), P = 0.297) and IL-17 (Mann-Whitney; 28.49 (0.00-28.49) versus 8.59 (0.00-8.59), P = 0.714) did not significantly differ between the 2 groups. These results confirm the important role of TNF-α in the pathogenesis of nail psoriasis and may suggest that anti-TNF agents could be more beneficial in psoriatic nail disease than agents targeting IL-12/23 p40 or IL-17 and its receptors.

Correlation of Specific Inflammatory Markers With the Occurrence of Depression in Patients With Psoriasis and Their Use as Biomarkers for the Diagnosis of Depression.

INTRODUCTION: Psoriasis is a systemic disease of the skin and nails associated with a wide range of comorbidities such as depression, psoriatic arthritis and metabolic syndrome. OBJECTIVES: The study aimed to examine a potential association between inflammatory markers (C- reactive protein [CRP] and erythrocyte sedimentation rate [ESR]) and depression in patients with psoriasis. METHODS: A total of 80 individuals were enrolled in the study. Case participants included 28 patients diagnosed with Psoriasis (Beck Depression Inventory-II: :0-13) and 24 patients diagnosed with Psoriasis and Depression (Beck Depression Inventory-II:14-63). Twenty-eight (28) healthy participants comprised the control group.Psoriasis severity was evaluated by using Psoriasis Area and Severity Index, Physician Global Assessment, Body Surface Area and Dermatology Life Quality Index. Written approval was obtained for its use in this study: Cardiff University (09/2015). Other factors considered in the study were obesity using the Body Mass Index, the levels of stress using the Beck Anxiety Inventory, and the presence of insomnia using the Athens Insomnia Scale. Blood draws and inflammatory markers measurements were performed for all participants. RESULTS: Both CRP and ESR levels were higher in the case group (ie Psoriasis and Depression and Psoriasis) compared to healthy controls. Furthermore, psoriatic patients with depression showed increased CRP and ESR levels compared to those of psoriatic patients without depression. CONCLUSIONS: The evaluation of both CRP and ESR and their use to detect the presence of depression in patients with psoriasis can be an important tool for their holistic treatment of theirs.

Differences among Indian and European pemphigus patients based on demographics, clinical parameters and propensity for relapse: results of a prospective bicontinental cohort study.

Limited studies have explored pemphigus variations among different ethnic groups residing in their respective geographical locations. This bicontinental study aimed to compare clinical and immunological parameters in Indian and European pemphigus patients in complete remission, off therapy, or on minimal therapy. 105 patients (India, n= 75; Bulgaria, n=15; Greece, n=15) with pemphigus vulgaris (PV) or pemphigus foliaceous (PF) in complete remission on minimal therapy (n=64) or complete remission off therapy (n=41) were recruited. Demographic, clinical, and immunological parameters were compared. Indian patients were significantly younger, the maximal disease severity during the preceding active disease phase was significantly lower, and treatment duration until complete remission was significantly shorter, compared to European patients. European patients had significantly higher anti-Dsg3 serum levels and higher IgG positivity rate based on direct immunofluorescence microscopy at baseline. Furthermore, European patients revealed higher CD19, CD19+ CD27+ cell counts, compared with patients from India. Of note, none of the European patients (n=30) relapsed within the study period, in contrast to 29/75 (38.6%) Indian patients. Treatment strategies differed significantly between the two cohorts, with more frequent utilization of rituximab to achieve remission in the Indian cohort, while prednisolone was more widely used for maintaining remission in the European cohort. The observed heterogeneity of pemphigus among patients of different ethnicities in terms of demographics, clinical parameters, and propensity for relapse may be due to genetic background or different treatment strategies.

Association of autoantibodies to BP180 with disease activity in Greek patients with bullous pemphigoid.

39 bullous pemphigoid (BP) patients were studied to assess the clinical significance of anti-BP180 and anti-BP230 circulating autoantibodies of BP and correlate their titers with the clinical scores of the BP Disease Area Index (BPDAI) and the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) as well as with the intensity of pruritus measured by the BPDAI pruritus component. All parameters were evaluated by the time of diagnosis (baseline), month 3, and month 6. Titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (r = 0.557, P value < 0.0001) and ABSIS (r = 0.570, P value < 0.0001) values, as well as with BPDAI component for the intensity of pruritus (rho = 0.530, P value = 0.001) at baseline. At month 3, titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (rho = 0.626, P value = 0.000) and ABSIS (rho = 0.625, P value = 0.000) values, as well as with the BPDAI component for the intensity of pruritus (rho = 0.625, P value = 0.000). At month 6, titers of anti-BP180 autoantibodies were strongly correlated with BPDAI (rho = 0.527, P value = 0.001) and ABSIS (rho = 0.526, P value = 0.001) values, as well as with the BPDAI component for the intensity of pruritus (rho = 0.525, P value = 0.001). There was no statistically significant correlation between titers of anti-BP230 autoantibodies and the BPDAI, ABSIS, and BPDAI component for the intensity of pruritus at the same time points.

Don't worry about a thing every little thing gonna be all right (except for acral lentiginous melanoma).

Bob Marley was a Jamaican singer, songwriter, and musician, considered one of the pioneers of reggae. In July 1977, he was diagnosed with acral lentiginous melanoma on his right great toe, which presented as a pigmented subungual lesion. Marley was advised to have his digit amputated, but he refused and opted for less invasive solutions. Unfortunately, he died at the age of 36 of metastatic disease in May 1981 after a 4-year battle with the disease. Marley has served as the posthumous poster child for skin cancer in people with skin of color for decades and has raised public awareness of this rare form of malignant melanoma.

Investigation of comorbid autoimmune diseases in women with autoimmune bullous diseases: An interplay of autoimmunity and practical implications.

Autoimmune bullous diseases are a group of skin disorders resulting from an autoimmune reaction against intercellular adhesion molecules or components of the basement membrane of skin and mucosa. Autoimmune disorders often occur in patients with a history of another autoimmune disease and most autoimmune diseases have a striking female predominance. In this review, we aim to analyze the different associations of autoimmune bullous diseases with other autoimmune diseases and highlight the distinctiveness of the female gender in these associations.

Fungal Infections and Nail Psoriasis: An Update.

The relationship between psoriasis and onychomycosis is controversial, and the exact nature of this association remains to be clearly elucidated. In healthy nails, the compact nail plate acts as a barrier, preventing any infection. In psoriatic nails, the nail plate involvement, together with abnormalities in the blood capillaries, may lead to decreased natural defenses against microorganisms. Moreover, onycholysis (detachment of the nail plate) induces a humid environment that may favor fungal proliferation. Treatment with immunosuppressive drugs may additionally enhance onychomycosis. In this comprehensive review, we present data regarding the incidence and pathogenic action of dermatophytes and other fungi in the development of fungal infection in psoriatic nails.

Detailed analysis of specific nail psoriasis features and their correlations with clinical parameters: a cross-sectional study.

BACKGROUND: Occurrence rates of specific features of psoriatic nails, as well as the influence of variable clinical parameters on nail involvement in psoriasis, are not determined. OBJECTIVE: To evaluate the frequency and characteristics of nail involvement in patients with psoriasis and determine the relationship between nail psoriasis and clinical parameters (age, gender, family history, clinical type, age of onset, duration, joint involvement). METHODS: 228 psoriatic patients, who had not received any systematic or topical antipsoriatic treatment for at least a year, were consecutively selected to participate in this cross-sectional study. RESULTS: 66.7% of patients had nail psoriasis. A logistic regression model showed that none of the clinical covariates were statistically significant in predicting nail psoriasis. CONCLUSION: The majority of psoriatic patients presented nail psoriasis. The most common feature was oil drop. There was a difference in the prevalence of each feature between fingernails and toenails. In correlation with clinical parameters, nail psoriasis evolves independently.

Accuracy and correlates of visual and verbal instruments assessing maternal perceptions of children's weight status: the Healthy Growth Study.

OBJECTIVE: To examine the accuracy of maternal ability to classify their children's weight status correctly using a verbal and a visual classification instrument and to detect significant correlates of maternal misperceptions. DESIGN: Cross-sectional study. SETTING: Primary schools in four counties from north, west, central and south Greece. SUBJECTS: A representative sample of 1858 primary-school children aged 9-13 years was examined. Two different instruments to assess maternal perceptions of their children's weight status, i.e. a verbal and a visual one, were used. RESULTS: Verbal and visual maternal underestimation rates of children's weight status were 15·0 % and 41·3 %, respectively. The frequency of underestimation was much higher among overweight and obese children for both instruments. The highest underestimation rates of 87·9 % and 82·1 % in overweight and obese boys, respectively, were obtained with the visual instrument. Multiple logistic regression modelling revealed that the likelihood of both verbal and visual maternal underestimation of their children's weight status was significantly higher for overweight mothers and for those with a lower educational level. Furthermore, children's male gender and a nanny or someone other than the mother as the child's primary caregiver were found to increase the odds of visual and verbal maternal underestimation of children's weight status, respectively. CONCLUSIONS: The present study showed that the verbal instrument used to assess maternal perceptions of their children's weight status was more accurate compared with the visual one. However, both instruments showed that a considerable number of overweight and obese boys had their weight status underestimated by their mothers. Educating mothers to classify their children's weight status correctly might be a key factor for the implementation of successful childhood obesity prevention initiatives.