Incidence of pemphigoid diseases in Northern Germany in 2016 - first data from the Schleswig-Holstein Registry of Autoimmune Bullous Diseases.

BACKGROUND: Autoimmune bullous diseases (AIBD) are rare disorders characterized by autoantibody formation against components of adhesion molecules; in pemphigoid diseases (PD), these are proteins of hemidesmosomes and basement membrane, important for cell-matrix adhesion in skin and/or mucous membranes. Incidences of these diseases vary considerably between different populations. OBJECTIVES: To establish a registry prospectively recruiting all AIBD patients in a geographically well-defined region in Northern Germany (Schleswig-Holstein). METHODS: Only patients with verified disease (by clinical presentation, histology, direct and/or indirect immunofluorescence and /or ELISA) living in Schleswig-Holstein were included. Incidences of PD were estimated based on the total number of inhabitants in Schleswig-Holstein, stratified by birth year and sex. RESULTS: Of 67 patients with PD [35 male, 32 female, mean age 75 (standard deviation 14.3 years)], 83% were patients with bullous pemphigoid [n = 56, 28 male, 28 female, mean age 78 (SD 9.9)]. The resulting crude incidences were 23.4 patients/million/year for all pemphigoid patients, 19.6 patients/million/year for bullous pemphigoid (age-standardized 16.9 patients/million/year) with a strong increase in bullous pemphigoid patients in the age group of 85-90 years with 262 patients/million/year. Incidences for bullous pemphigoid were higher in urban compared to rural areas. Other PD (mucous membrane pemphigoid, linear IgA disease, anti-p200 pemphigoid) were less frequent with crude incidences of 2.1, 1.0 and 0.7 patients/million/year, respectively. CONCLUSIONS: This study prospectively analyses the incidence of PD in a carefully defined geographical area. The highest incidence among PD patients was found for bullous pemphigoid. The incidence of bullous pemphigoid is considerably increased compared to previous reports and reveals regional differences. Further studies are needed in order to clarify these findings.

[Epidermolysis bullosa acquisita]. / Epidermolysis bullosa acquisita.

Epidermolysis bullosa acquisita (EBA) is a rare acquired subepidermal bullous autoimmune dermatosis, associated with autoantibodies against collagen type VII, the most important component of dermal anchoring fibrils. Blister induction occurs after binding of autoantibodies to collagen type VII, leading to complement activation, recruitment of neutrophils and secretion of proteases. Clinically, the disease is mostly characterized by tense blisters on trauma-exposed body areas which heal with scarring (mechanobullous form of EBA). The second most frequent subtype of EBA is inflammatory EBA, a bullous pemphigoid-like disease associated with pruritus. Involvement of mucous membranes and/or lesions in the head and neck area additionally point to the diagnosis of EBA. The mechanobullous type of EBA and EBA with intensive mucous membrane lesions display a chronic course and are often extremely resistant to therapy. Topical and systemic glucocorticoids, dapsone, colchicine, classical immunosuppressants, anti-CD20 antibodies, immunoadsorption or intravenous immunoglobulins have been reported as treatments.

Severity of polymicrobial sepsis modulates mitochondrial function in rat liver.

Mitochondrial dysfunction is assumed to be an important contributor to multi organ dysfunction syndrome. Here, the effects of varying degrees of sepsis on hepatic mitochondrial function were investigated. Moderate or more severe sepsis was induced in rats using a colon ascendens stent peritonitis (CASP)-model (16 G and 14 G stent respectively). Respiratory control ratio (RCR) was significantly higher in the 16 G-group and unchanged in the 14 G-group compared with healthy controls. The ADP/O ratio was similar in all groups. Our results indicate that different severities of sepsis differently influence the mitochondrial function, which could be a sign of adaptive reaction.

Pseudoentanglement of spin states in the multilevel 15N@C60 system.

We have prepared combined electron and nuclear spin pseudoentangled states Psi+/-27 and Phi+/-18 out of the total number of eight quantum states in the multilevel quantum system of a nitrogen atom with electron spin 3/2 and nuclear spin 1/2 encaged in the endohedral fullerene (15)N@C(60). Density matrix tomography was applied to verify the degree of entanglement.

Electron paramagnetic resonance investigation of endohedral fullerenes N@C(60) and N@C(70) in a liquid crystal.

Endohedral fullerenes N@C(60) and N@C(70) were dissolved in the liquid crystal 4-methoxybenzylidene-4'-n-butylaniline (MBBA) and investigated by electron paramagnetic resonance. In both cases well resolved EPR spectra give proof for molecular orientation in the nematic mesophase. Spectral features are dominated by a nonvanishing zero-field interaction, indicating a deviation from spherical spin density distribution at the encased nitrogen atom. In N@C(70), a maximum order parameter O(33) = 0.18(3), correlated with the long axis of the cage, and a zero-field-splitting parameter D = -2.6(4) MHz were determined. A persistent zero-field splitting is also observed in C(60) via the quartet spin of the encapsulated nitrogen, although no assignment of the director with respect to the molecular frame is possible. The observed line splitting is indicative of pseudo orientation of the rapidly rotating cage in this case.

Experimental confirmation of the predicted shallow donor hydrogen state in zinc oxide.

We confirm the recent prediction that interstitial protium may act as a shallow donor in zinc oxide, by direct spectroscopic observation of its muonium counterpart. On implantation into ZnO, positive muons--chemically analogous to protons in this context--form paramagnetic centers below about 40 K. The muon-electron contact hyperfine interaction, as well as the temperature and activation energy for ionization, imply a shallow level. Similar results for the cadmium chalcogenides suggest that such shallow donor states are generic to the II-VI compounds. The donor level depths should serve as a guide for the electrical activity of interstitial hydrogen.