Hana Jedličková

Cutaneous lupus erythematosus: First multicenter database analysis of 1002 patients from the European Society of Cutaneous Lupus Erythematosus (EUSCLE)

In this prospective, cross-sectional, multicenter study, we assessed clinical and laboratory characteristics from patients with cutaneous lupus erythematosus (CLE) using the Core Set Questionnaire of the European Society of Cutaneous Lupus Erythematosus (EUSCLE). 1002 (768 females, 234 males) patients with different subtypes of CLE, such as acute CLE (ACLE, 304 patients), subacute CLE (SCLE, 236 patients), chronic CLE (CCLE, 397 patients), and intermittent CLE (ICLE, 65 patients), from 13 European countries were collected and statistically analyzed by an SPSS database. The main outcome measures included gender, age at onset of disease, LE-specific and LE-nonspecific skin lesions, photosensitivity, laboratory features, and the criteria of the American College of Rheumatology (ACR) for the classification of systemic lupus erythematosus. The mean age at onset of disease was 43.0±15.7 years and differed significantly between the CLE subtypes. In 347 (34.6%) of the 1002 patients, two or more CLE subtypes were diagnosed during the course of the disease and 453 (45.2%) presented with LE-nonspecific manifestations. Drug-induced CLE and Sjögrens Syndrome had the highest prevalence in SCLE patients (13.1% and 14.0%, respectively). Photosensitivity was significantly more frequent in patients with ACLE, SCLE, and ICLE compared with those with CCLE. The detection of antinuclear antibodies such as anti-Ro/SSA and anti-La/SSB antibodies revealed further significant differences between the CLE subtypes. In summary, the EUSCLE Core Set Questionnaire and its database facilitate the analysis of clinical and laboratory features in a high number of patients with CLE and will contribute to standardized assessment and monitoring of the disease in Europe.

Bullous pemphigoid and internal diseases – A case-control study

To study associations of bullous pemphigoid (BP) with internal diseases, we conducted a retrospective case control study assessing the frequency of selected diseases - diabetes mellitus, neurological diseases, malignant tumors, benign prostate hyperplasia, hypertension and ischemic heart disease in patients with BP. 89 patients with BP, whose data were retrieved from the register of the Centre of bullous diseases from the period of 1991-2006, were matched with 89 controls of the same age and gender, recruited from patients treated for other skin diseases. The frequency of internal diseases at the time of the onset of BP was evaluated by unconditional logistic regression adjusted for age and gender and maximum likelihood test for contingency tables. Neurological disease was found in 42.7% of the patients and in 19.1% of controls. This difference was statistically significant (p value = 0.001). Moreover, regression analysis has shown that patients with neurological disease in the age group >or= 80 years have significantly higher risk of pemphigoid than patients without neurological disease (odds ratio 10.55; 95% confidence interval 2.68 to 41.49). Most frequent were cerebral stroke in men and dementia in women. For other diseases and other age groups, no statistically significant influence was found.

Management of bullous pemphigoid: the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology

Bullous pemphigoid is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or generalized bullous lesions. In up to 20% of affected patients, bullae may be completely absent, and only excoriations, prurigo‐like lesions, eczematous lesions, urticated lesions and/or infiltrated plaques are observed. The disease is significantly associated with neurological disorders. The morbidity of bullous pemphigoid and its impact on quality of life are significant. So far, a limited number of national treatment guidelines have been proposed, but no common European consensus has emerged. Our consensus for the treatment of bullous pemphigoid has been developed under the guidance of the European Dermatology Forum in collaboration with the European Academy of Dermatology and Venereology. It summarizes evidence‐based and expert‐based recommendations.

Brunsting-Perry pemphigoid of the scalp with antibodies against laminin 332.

In this report, we describe 2 patients with localized CP of the scalp and IgG auto-antibodies against laminin 332.

Diagnosis and Management of Pemphigus: recommendations by 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 recommendations. METHODS A preliminary survey, based on the European Dermatology Forum (EDF) and the European Academy of Dermatology and Venereology (EADV) guidelines, was sent to a panel of international experts to determine the level of consensus. The results were discussed at the International Bullous Diseases Consensus Group in March 2016 during the annual American Academy of Dermatology (AAD) conference. A second survey was sent following the meeting to more experts to achieve greater international consensus. RESULTS The 39 experts participated in the first round of the Delphi-survey while 54 from 21 countries completed the second round. The number of statements in the survey was reduced from 175 topics in Delphi I to 24 topics in Delphi II based on Delphi results and meeting discussion. LIMITATIONS Each recommendation represents the majority opinion and therefore may not reflect all possible treatment options available. CONCLUSIONS We present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first line therapy option for moderate to severe pemphigus.

Serological diagnostics in the detection of IgG autoantibodies against human collagen VII in epidermolysis bullosa acquisita – a multicenter analysis

Epidermolysis bullosa acquisita (EBA) is a rare, potentially devastating autoimmune disease of the skin. IgG autoantibodies directed against type VII collagen (Col7), the major component of anchoring fibrils, induce skin fragility leading to cutaneous and mucocutaneous blister formation, which is mostly of a scarring phenotype. Thus, powerful and reproducible diagnostic assays are critical to establish the diagnosis of EBA early to avoid irreversible sequelae.

Dystrophic epidermolysis bullosa pruriginosa with autoantibodies against collagen VII

ejd.2012.1709 Auteur(s) : Hana JEDLICKOVA1 hana.jedlickova@fnusa.cz, Ralf MULLER2,a, Daniele CASTIGLIA3,a, Milica KOVACEVIC4, Josef FEIT5 1 Dept. of Dermatovenereology, St. Anna University Hospital, Pekarska 53, 65691 Brno, Czech Republic 2 Dept. of Dermatology, Philipps University,35043 Marburg, Germany 3 Laboratory of Molecular and Cell Biology -IRCCS, 00167 Rome, Italy 4 Dept. of Dermatovenereology, 5 Dept. of Pathology, University Hospital Brno, 62500 Brno, Czech Republic a These authors contributed [...]

Hypertext atlas of dermatopathology with expert system for epithelial tumors of the skin

Background:  Image resolution required for reference histological images is high. Obtaining high‐resolution images requires a system, composing large image from individual smaller components. Such systems must thus be capable of automatically taking individual image parts, performing shading compensation and fusion of the image parts into one large image. Distribution of such images over the Internet requires developing a suitable users interface with access to the image details.

Bullous systemic lupus erythematosus induced by the therapy for lupus nephritis

Je popsan připad mladeho muže s buloznim SLE. Bulozni leze vznikly při zahajeni pulsni terapie lupusove nefritidy methylprednisolonem, cyklofosfamidem a mesnou. Bulozni SLE je vzacne onemocněni s tvorbou autoprotilatek proti kolagenu VII. Je uvažovano o asociaci s lupusovou nefritidou. V nasem připadě se puchýřnate onemocněni zhojilo lokalni terapii a pulsni terapie pokracovala v nižsich davkach bez dalsich nežadoucich ucinků.

Virtual microscope interface to high resolution histological images

The Hypertext atlas of Dermatopathology, the Atlas of Fetal and Neonatal Pathology and Hypertext atlas of Pathology (this one in Czech only) are available at http://www.muni.cz/atlases. These atlases offer many clinical, macroscopic and microscopic images, together with short introductory texts. Most of the images are annotated and arrows pointing to the important parts of the image can be activated.The Virtual Microscope interface is used for the access to the histological images obtained in high resolution using automated microscope and image stitching, possibly in more focusing planes. Parts of the image prepared in advance are downloaded on demand to save the memory of the users computer. The virtual microscope is programmed in JavaScript only, works in Firefox/Mozilla and MSIE browsers without need to install any additional software.