Efficacy and safety of rituximab in pemphigus: experience of the German Registry of Autoimmune Diseases.

Background: Rituximab has been reported to be effective in various small case series of patients with severe and/or refractory pemphigus. However, no systematic evaluation is available to corroborate this observation. The aim of this study was to systematically determine efficacy and safety of rituximab in treatment-resistant pemphigus.

Immunosuppressive therapy for autoimmune bullous diseases.

Adjuvant immunosuppressive drugs are widely used to minimize corticosteroid-related adverse effects in the short-term and long-term management of cautoimmune bullous diseases. In bullous pemphigoid and pemphigus vulgaris, azathioprine and mycophenolate mofetil seem to be equally effective when used in combination with oral corticosteroids, but mycophenolate mofetil is less myelosuppressive and hepatotoxic. Due to a better safety profile, mycophenolate mofetil or enteric-coated mycophenolate sodium may gradually replace azathioprine as the first-line adjuvant of choice in the treatment of moderate to severe autoimmune bullous diseases, including epidermolysis bullosa acquisita and cicatricial pemphigoid.

Efficacy of pimecrolimus 1% cream in the long term management of atopic hand dermatitis. A double-blind RCT.

Background: Efficacy and steroid sparing effects of pimecrolimus 1% cream in atopic dermatitis have been shown recently, but there is no data on efficacy in long term management of atopic hand dermatitis. This study aims to investigate the efficacy of pimecrolimus 1% cream as maintenance therapy in patients suffering from atopic hand dermatitis.

Patients And Methods: A double-blind vehicle controlled study in 40 adult patients with atopic hand dermatitis (IGA ≤ 3) comparing the efficacy of twice daily application of pimecrolimus 1% cream given as maintenance treatment versus vehicle over a 8 week period after clinical response (IGA ≤ 2) to a 1-3 week pre-treatment with mometasone fuorate 0.

Definitions and outcome measures for bullous pemphigoid: recommendations by an international panel of experts.

Our scientific knowledge of bullous pemphigoid (BP) has dramatically progressed in recent years. However, despite the availability of various therapeutic options for the treatment of inflammatory diseases, only a few multicenter controlled trials have helped to define effective therapies in BP. A major obstacle in sharing multicenter-based evidences for therapeutic efforts is the lack of generally accepted definitions for the clinical evaluation of patients with BP.

Frequency of disease-associated and other nuclear autoantibodies in patients of the German Network for Systemic Scleroderma: correlation with characteristic clinical features.

Introduction: In the present study, we analysed in detail nuclear autoantibodies and their associations in systemic sclerosis (SSc) patients included in the German Network for Systemic Scleroderma Registry.

Methods: Sera of 863 patients were analysed according to a standardised protocol including immunofluorescence, immunoprecipitation, line immunoassay and immunodiffusion.

Results: Antinuclear antibodies (ANA) were detected in 94.

Evaluation of the atrophogenic potential of hydrocortisone 1% cream and pimecrolimus 1% cream in uninvolved forehead skin of patients with atopic dermatitis using optical coherence tomography.

Topical corticosteroids are widely used to treat atopic dermatitis (AD), but their anti-inflammatory mode of action can be accompanied by several unwanted side effects including skin atrophy and telangiectasia. In this 8-week, investigator-blinded, intraindividual right-left comparison study with patients with mild-to-moderate AD, hydrocortisone 1% cream (HCT) was applied twice daily for 4 weeks on one side of forehead skin without clinical signs of AD and pimecrolimus 1% cream (PIM) on the other. Epidermal and dermal thickness were assessed by optical coherence tomography (OCT) and high-frequency ultrasound, respectively.

Up-regulation of the chemokine CCL18 by macrophages is a potential immunomodulatory pathway in cutaneous T-cell lymphoma.

Mycosis fungoides (MF) is the most frequent form of cutaneous T-cell lymphoma (CTCL), which can deteriorate from patch stage to dermal-based tumors and systemic involvement in years. The interaction of chemokines in the skin with CTCL cells might have implications for the pathogenesis of the disease. In this study, we show by PCR analysis and immunofluorescence staining that the chemokine CCL18 is present in skin biopsy specimens of patients with MF and its precursor form parapsoriasis en plaque but not in healthy tissue.

Efficacy and safety of omalizumab in patients with chronic urticaria who exhibit IgE against thyroperoxidase.

Background: A subgroup of patients with chronic spontaneous urticaria (CU) exhibits IgE antibodies directed against autoantigens, such as thyroperoxidase (TPO). We conducted this study to investigate whether such patients with CU with IgE against TPO benefit from treatment with omalizumab, a humanized anti-IgE mAb licensed for the treatment of severe persistent allergic (IgE-mediated) asthma.

Objectives: We sought to assess the efficacy of omalizumab treatment in patients with CU with IgE autoantibodies against TPO.

Safety and clinical outcomes of rituximab therapy in patients with different autoimmune diseases: experience from a national registry (GRAID).

Introduction: Evidence from a number of open-label, uncontrolled studies has suggested that rituximab may benefit patients with autoimmune diseases who are refractory to standard-of-care. The objective of this study was to evaluate the safety and clinical outcomes of rituximab in several standard-of-care-refractory autoimmune diseases (within rheumatology, nephrology, dermatology and neurology) other than rheumatoid arthritis or non-Hodgkin's lymphoma in a real-life clinical setting.

Methods: Patients who received rituximab having shown an inadequate response to standard-of-care had their safety and clinical outcomes data retrospectively analysed as part of the German Registry of Autoimmune Diseases.

Human slan (6-sulfo LacNAc) dendritic cells are inflammatory dermal dendritic cells in psoriasis and drive strong TH17/TH1 T-cell responses.

Background: Psoriasis is a chronic inflammatory skin disease that is considered to result from activated T cells stimulated by a population of inflammatory dermal dendritic cells (DCs). The origin and identity of these inflammatory dermal DCs are largely unknown.

Objective: We previously identified slanDCs (6-sulfo LacNAc) DCs as a rich source of TNF-α and as the early major source of IL-12.

Addition of pimecrolimus cream 1% to a topical corticosteroid treatment regimen in paediatric patients with severe atopic dermatitis: a randomized, double-blind trial.

Background: Pimecrolimus and topical corticosteroids (TCS) combination therapy may provide an alternative treatment for patients with severe atopic dermatitis (AD), with faster clearance of disease flares, consequently reducing the duration of TCS treatment.

Objective: To assess the safety profile of pimecrolimus cream 1% combined with fluticasone versus fluticasone alone in paediatric patients with severe AD.

Methods: Patients (n = 376) were randomized to a combination of pimecrolimus cream 1% with fluticasone or vehicle plus fluticasone for 4 weeks.

Conversion of vitamin D3 to hormonally active 1alpha,25-dihydroxyvitamin D3 in cultured keratinocytes: relevance to cell growth and differentiation.

The regulatory potential of intracellularly generated calcitriol on growth and differentiation of cultured keratinocytes is determined by the degree of cell confluence and availability of the highly lipophilic substrate vitamin D3 to these cells. The enzymatic conversion of vitamin D3 to calcitriol is considerably elevated in the presence of the nontoxic surfactant copolymer pluronic F127 (120 microg/ml medium) compared to the control without this agent. We found a positive correlation between the formation rate of calcitriol and inhibition of the 3H-thymidine incorporation rate into the DNA of keratinocytes.

Vitamin D metabolism.

Irradiation of human skin with ultraviolet B (280-320 nm) initiates the photochemical conversion of 7-dehydrocholesterol via previtamin D3 to vitamin D3. Vitamin D3 needs for its activation two hydroxylation steps in the liver and kidney. The final product, hormonally active 1alpha,25-dihydroxyvitamin D3 (calcitriol), arrives via the circulation to its target tissues and acts in a genomic or nongenomic manner.

Immunoadsorption in the treatment of pemphigus.

The autoimmune bullous skin disorder pemphigus is characterized by the presence of autoantibodies that target distinct adhesion molecules of the epidermis. The autoantibodies result in a loss of the epidermal adhesive properties, which in turn leads to the appearance of blisters and erosions on skin and mucosal surfaces. In the majority of cases, disease activity can be sufficiently controlled by systemic corticosteroids in combination with immunsuppressants, but pemphigus often is recalcitrant to conventional immunosuppressive therapy.

Lipidome of narrow-band ultraviolet B irradiated keratinocytes shows apoptotic hallmarks.

Background: UV light triggers a variety of biological responses in irradiated keratinocytes that might be associated with global perturbation of their lipidome. However, lipids that are specifically affected and the exact molecular mechanisms involved remain poorly understood.

Objectives: To characterize time-dependent changes of the lipidome of cultured keratinocytes induced by narrow-band ultraviolet B (NB-UVB) irradiation.

Early exposure to antibiotics and infections and the incidence of atopic eczema: a population-based cohort study.

It has been suggested that infants exposed to antibiotics are at increased risk for atopic eczema (AE), whereas the early exposure to infections might be protective. This study describes the complex relationship between early exposure to infections, anti-infectious treatment with antibiotics, and incident AE. Using a German population-based administrative health-care and prescription database, we established a cohort of 370 children not diagnosed as having AE during their first year of life.

Effects of the introduction of the German "Praxisgebühr" on outpatient care and treatment of patients with atopic eczema.

Background: The introduction of a co-payment of 10 Euros per quarter and physician for adults (the so called "Praxisgebühr") as of January 01, 2004 was a significant health policy measure with unknown effects on medical care of patients with atopic eczema (AE).

Methods: Analysis of an administrative healthcare database from Saxony, Germany. Comparison of outpatient care and treatment of 11,036 patients with AE (6,696 adults) in the year before (2003) and after (2004) the introduction of the co-payment using descriptive statistics and logistic regression modeling.

Use of high-dose immunoglobulins in dermatology.

The treatment of severe autoimmune skin diseases and of toxic epidermal necrolysis (ICD: L51.2) with high-dose intravenous immunoglobulins (IVIg) is an established therapeutic procedure in dermatology. As IVIg are usually only administered in rare autoimmune diseases or in particularly severe disease courses, use of immunoglobulins in dermatology is commonly not based on experience from controlled and randomized studies typically demanded by evidence-based medicine.

Sporotrichoid atypical cutaneous infection caused by Mycobacterium marinum.

A case of a sporotrichoid cutaneous infection caused by Mycobacterium marinum is reported. A 53- year-old male patient presented with red, partly purulent nodular lesions on the back of his left hand, forearm, and upper medial arm that had developed consecutively during the past 4 weeks. A mycobacterial infection with M.

High frequency of corticosteroid and immunosuppressive therapy in patients with systemic sclerosis despite limited evidence for efficacy.

Introduction: In systemic sclerosis (SSc) little evidence for the effectiveness of anti-inflammatory and immunosuppressive therapy exists. The objective of this study was to determine the extent to which SSc patients are treated with corticosteroids and immunosuppressive agents.

Methods: Data on duration and dosage of corticosteroids and on the type of immunosuppressive agent were analyzed from 1,729 patients who were registered in the German Network for Systemic Scleroderma (DNSS).

Narrow-band UVB-induced externalization of selected nuclear antigens in keratinocytes: implications for lupus erythematosus pathogenesis.

The aim of this study was to analyze whether sera obtained from patients with lupus erythematosus (LE) react with membrane structures found on keratinocytes irradiated with narrow-band ultraviolet B (NB-UVB). We applied atomic force microscopy (AFM) to visualize cell surface structures expressing nuclear antigens upon apoptosis following NB-UVB irradiation. Immortalized human keratinocytes (HaCaT) were cultured under standard conditions, irradiated with 800 mJ cm(-2) NB-UVB light and imaged by AFM mounted on an inverted optical microscope.