Dupilumab as a novel therapy for bullous pemphigoid: A multicenter case series.

Background: Bullous pemphigoid (BP) is an autoimmune blistering disorder occurring mostly in the elderly that lacks adequate treatments.

Objective: To describe our experience using dupilumab in a series of patients with BP.

Methods: This is a case series of patients from 5 academic centers receiving dupilumab for BP.

Diagnosis and management of pemphigus: Recommendations of an international panel of experts.

Background: Several European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management.

Objective: We now present results from a subsequent Delphi consensus to broaden the generalizability of the recommendations.

Methods: A preliminary survey, based on the European Dermatology Forum and the European Academy of Dermatology and Venereology guidelines, was sent to a panel of international experts to determine the level of consensus.

Case of Pediatric Bullous Systemic Lupus Erythematosus Treated with Intravenous Immunoglobulin.

This is a case report of a 16-year-old girl recently diagnosed with systemic lupus erythematosus (SLE) who presented with multiple blisters on the face, hands, arms, legs, trunk, and vaginal and oral mucosa. Skin biopsy was consistent with bullous SLE (BSLE). Dapsone is often the first-line treatment option for BSLE, but the patient's history of anemia and leukopenia and long-term immunosuppression requirement for her systemic symptoms raised concerns about dapsone and bone marrow toxicity, especially hemolytic anemia and agranulocytosis.

Paraneoplastic pemphigus: a short review.

Paraneoplastic pemphigus (PNP) is a fatal autoimmune blistering disease associated with an underlying malignancy. It is a newly recognized blistering disease, which was first recognized in 1990 by Dr Anhalt who described an atypical pemphigus with associated neoplasia. In 2001, Nguyen proposed the term paraneoplastic autoimmune multiorgan syndrome because of the recognition that the condition affects multiple organ systems.

Alopecia areata profiling shows TH1, TH2, and IL-23 cytokine activation without parallel TH17/TH22 skewing.

Background: Alopecia areata (AA) is a common T cell-mediated disorder with limited therapeutics. A molecular profile of cytokine pathways in AA tissues is lacking. Although studies have focused on TH1/IFN-γ responses, several observations support a shared genetic background between AA and atopy.

Intravenous immunoglobulin in the treatment of autoimmune bullous dermatoses: an update.

High-dose intravenous immunoglobulin (IVIg) is being increasingly utilized as an off-label therapy for a variety of autoimmune and inflammatory conditions across various specialties. Numerous reports have shown that it is an effective treatment for autoimmune skin blistering disorders. Unlike most therapies for blistering disorders, IVIg is not immunosuppressive and has a favorable side effect profile.

Paraneoplastic autoimmune multiorgan syndrome: 20 years after.

The purpose of this review is to provide insight and clarification in the quandary of classification and delineate clinical and histological features and pathophysiology of paraneoplastic pemphigus. This is a paraneoplastic disease of epithelial autoimmunity and adhesion originally described by Dr. Anhalt in 1990.

The cross-sectional GRAS sample: a comprehensive phenotypical data collection of schizophrenic patients.

Background: Schizophrenia is the collective term for an exclusively clinically diagnosed, heterogeneous group of mental disorders with still obscure biological roots. Based on the assumption that valuable information about relevant genetic and environmental disease mechanisms can be obtained by association studies on patient cohorts of ≥ 1000 patients, if performed on detailed clinical datasets and quantifiable biological readouts, we generated a new schizophrenia data base, the GRAS (Göttingen Research Association for Schizophrenia) data collection. GRAS is the necessary ground to study genetic causes of the schizophrenic phenotype in a 'phenotype-based genetic association study' (PGAS).

Effect of intravenous immunoglobulin with or without cytotoxic drugs on pemphigus intercellular antibodies.

Background: Intravenous immunoglobulin (IVIg)--a relatively new approach to treat pemphigus--lowers serum levels of pemphigus antibodies; however, the optimal way to use this agent is unknown.

Objective: We sought to examine whether coadministration of a cytotoxic drug to patients with pemphigus improves the ability of IVIg to decrease serum levels of intercellular (IC) antibodies.

Methods: In this retrospective study, we analyzed changes in IC antibody levels in 20 patients with pemphigus who were treated with 24 courses of IVIg administered alone (n = 10) or with a cytotoxic drug (n = 14).

Improvement of intravenous immunoglobulin therapy for bullous pemphigoid by adding immunosuppressive agents: marked improvement in depletion of circulating autoantibodies.

Background: Various antibody-mediated autoimmune disorders are treated with intravenous immunoglobulin (IVIg). While the exact action of IVIg is unknown, it likely acts to rapidly and selectively lower the level of pathogenic antibodies. The most effective use of IVIg, an expensive and potentially toxic treatment of autoimmune disorders, remains undetermined.

Intravenous immunoglobulin selectively decreases circulating autoantibodies in pemphigus.

Background: Autoantibody-mediated diseases such as pemphigus are caused by a single or very limited number of pathogenic autoantibodies. A major problem with all current therapies for these diseases is that they target all antibodies rather than selectively targeting only pathogenic antibodies. The following study was conducted to confirm observations made in a limited number of patients that suggest intravenous immunoglobulin (IVIg) may be able to selectively lower serum levels of only abnormal autoantibodies.

Improvement of cognitive functions in chronic schizophrenic patients by recombinant human erythropoietin.

Schizophrenia is increasingly recognized as a neurodevelopmental disease with an additional degenerative component, comprising cognitive decline and loss of cortical gray matter. We hypothesized that a neuroprotective/neurotrophic add-on strategy, recombinant human erythropoietin (rhEPO) in addition to stable antipsychotic medication, may be able to improve cognitive function even in chronic schizophrenic patients. Therefore, we designed a double-blind, placebo-controlled, randomized, multicenter, proof-of-principle (phase II) study.

Long-term citalopram administration reduces responsiveness of HPA axis in patients with major depression: relationship with S-citalopram concentrations in plasma and cerebrospinal fluid (CSF) and clinical response.

Rationale: A dysregulation of the hypothalamic-pituitary-adrenal (HPA) axis is a well-documented neurobiological finding in major depression. Moreover, clinically effective therapy with antidepressant drugs may normalize the HPA axis activity.

Objective: The aim of this study was to test whether citalopram (R/S-CIT) affects the function of the HPA axis in patients with major depression (DSM IV).

Multislice spiral computed tomographic angiography of coronary arteries in patients with suspected coronary artery disease: an effective filter before catheter angiography?

Background: Despite impressive image quality, it is unclear if noninvasive coronary angiography with multislice spiral computed tomography (CT) is powerful enough to act as a filter before invasive angiography (INV-A) in symptomatic patients.

Methods And Results: We therefore studied 133 consecutive symptomatic patients with suspected coronary artery disease (CAD) and an indication for INV-A (chest pain and signs of ischemia in conventional stress tests). Patients with known CAD, acute coronary syndrome, or a calcium volume score >1000 were excluded.

Neuropeptide Y and corticotropin-releasing hormone in CSF mark response to antidepressive treatment with citalopram.

Neuropeptides appear to play a role in the pathophysiology of depression and electroconvulsive treatment and lithium affect these compounds in human cerebrospinal fluid (CSF) and rodent brain. Consequently, we investigated whether long-term treatment with the selective serotonin reuptake inhibitor (SSRI) citalopram (Cit) would also affect neuropeptides in CSF of depressed patients. Changes in CSF monoamine metabolites were also explored.

Structural domains involved in the regulation of transmitter release by synapsins.

Synapsins are a family of neuron-specific phosphoproteins that regulate neurotransmitter release by associating with synaptic vesicles. Synapsins consist of a series of conserved and variable structural domains of unknown function. We performed a systematic structure-function analysis of the various domains of synapsin by assessing the actions of synapsin fragments on neurotransmitter release, presynaptic ultrastructure, and the biochemical interactions of synapsin.

Stereoselective metabolism of citalopram in plasma and cerebrospinal fluid of depressive patients: relationship with 5-HIAA in CSF and clinical response.

Plasma and cerebrospinal fluid (CSF) concentrations of the enantiomers of citalopram (CIT), its N-demethylated metabolite demethylcitalopram (DCIT) and its deaminated metabolite citalopram propionic acid derivative (CIT-PROP) were measured in plasma and CSF in 22 depressed patients after a 4-week treatment with 40 mg/d citalopram, which was preceded by a 1-week washout period. CSF 5-hydroxyindoleacetic acid (5-HIAA) and homovanillic acid (HVA) were measured at baseline and after the 4-week CIT medication period. Patients were assessed clinically, using the Hamilton Depression Rating Scale (21-item HAM-D): at baseline and then at weekly intervals.

Reliability and validity of Quality of Life assessed by the Short-Form 36 and the Modular System for Quality of Life in patients with schizophrenia and patients with depression.

Reliability and validity of the Quality of Life (QoL) construct were investigated in healthy controls (N=346), patients with depression (N=114) and patients with schizophrenia (N=91) using two different QoL instruments: the Short-Form 36 (SF-36), a well-established generic instrument measuring eight dimensions; and the Modular System for Quality of Life (MSQoL), a recently developed instrument measuring seven core dimensions and four specific modules (objective data, partnership, family, occupation). The MSQoL and the SF-36 were administered at three intervals (hospital admission, discharge and 4-month follow-up). Reliability, group profiles (clinical specificity), responsiveness, discriminant validity (with regard to sociodemographic, psychopathological, clinical and state variables) and convergent validity were tested.

[Invasive versus noninvasive (MSCT) coronary angiography. Importance of cardiac diagnostics with multislice computed tomography].

Cardiac imaging with fast computed tomography (CT) is a quickly evolving field starting to become established in the cardiac routine work-up. The exclusion of coronary calcification is the most accurate noninvasive method to exclude significant coronary stenosis whereas the detection of calcification identifies coronary arteriosclerosis. The total calcium load correlates with the risk of coronary stenosis, but there is not a 1 : 1 relationship.

[Personality and personality disorders II. The Specificity of the DAPP-model as a diagnostic system for personality disorders].

The 'Dimensional Assessment of Personality Pathology - Basic Questionnaire' (DAPP) measures 18 traits to provide a systematic representation of the overall domain of personality disorders (PD). The present study investigated the relationships between DAPP personality profiles and dimensional assessments of DSM-IV PD in general population subjects (n = 156), and a sample of 220 nonpsychotic psychiatric patients (including n = 67 PD patients). Using nonmetric multidimensional scaling models the similarities between the 18 DAPP-factors and the dimensional scores of the 12 DSM-IV PD (inclusive appendix) were graphically represented in a 2-dimensional similarity-system.