S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV).

Background: Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans.

International multicentre observational study to assess the efficacy and safety of a 0·5 mg kg per day starting dose of oral corticosteroids to treat bullous pemphigoid.

Background: European guidelines propose a 0·5 mg kg per day dose of oral prednisone as initial treatment for bullous pemphigoid (BP). We assessed the safety and efficacy of this regimen depending on BP extent and general condition of the patients.

Methods: In a prospective international study, we consecutively included all patients diagnosed with BP.

Oxidative Stress-Mediated YAP Dysregulation Contributes to the Pathogenesis of Pemphigus Vulgaris.

(PV) is a life-threatening autoimmune disease manifested with blisters in the skin and mucosa and caused by autoantibodies against adhesion protein desmoglein-3 (Dsg3) expressed in epithelial membrane linings of these tissues. Despite many studies, the pathogenesis of PV remains incompletely understood. Recently we have shown Dsg3 plays a role in regulating the yes-associated protein (YAP), a co-transcription factor and mechanical sensor, and constraining reactive oxygen species (ROS).

Multicenter prospective study on multivariant diagnostics of autoimmune bullous dermatoses using the BIOCHIP technology.

Background: The current standard in the serologic diagnosis of autoimmune bullous diseases (AIBD) is a multistep procedure sequentially applying different assays. In contrast, the BIOCHIP Mosaic technology combines multiple substrates for parallel analysis by indirect immunofluorescence.

Methods: Sera from 749 consecutive, prospectively recruited patients with direct immunofluorescence-positive AIBD from 13 international study centers were analyzed independently and blinded by using (1) a BIOCHIP Mosaic including primate esophagus, salt-split skin, rat bladder, monkey liver, monkey liver with serosa, recombinant BP180 NC16A, and gliadin GAF3X, as well as HEK293 cells expressing recombinant desmoglein 1, desmoglein 3, type VII collagen, and BP230 C-terminus and (2) the conventional multistep approach of the Department of Dermatology, University of Lübeck.

Alopecia and Hair Damage Induced by Oncological Therapy.

Damage and loss of hair (alopecia) is a predictable adverse event of oncological therapy. It can be caused by chemotherapy, radiotherapy, or targeted and hormonal therapy. From the point of view of patients with malignant disease, hair loss is one of the most feared side effects and adversely affects their mental health.

The desmosomal cadherin desmoglein-3 acts as a keratinocyte anti-stress protein via suppression of p53.

Desmoglein-3 (Dsg3), the Pemphigus Vulgaris (PV) antigen (PVA), plays an essential role in keratinocyte cell-cell adhesion and regulates various signaling pathways involved in the progression and metastasis of cancer where it is upregulated. We show here that expression of Dsg3 impacts on the expression and function of p53, a key transcription factor governing the responses to cellular stress. Dsg3 depletion increased p53 expression and activity, an effect enhanced by treating cells with UVB, mechanical stress and genotoxic drugs, whilst increased Dsg3 expression resulted in the opposite effects.

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.

Serological diagnostics in the detection of IgG autoantibodies against human collagen VII in epidermolysis bullosa acquisita: a multicentre analysis.

Background: 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.

Paraneoplastic Scleroderma: Are There Any Clues?

Dear Editor, Scleroderma associated with neoplasia is rare, with only a small number of cases reported. We describe 4 patients with paraneoplastic scleroderma who were treated at the I. Department of Dermatovenereology, St.

Measurement of transepidermal water loss in localized scleroderma.

Localized scleroderma (LS) is a disease characterized by fibrotic changes in the dermis. Connective tissue growth factor and transforming growth factor β2 are the main mediators of fibrogenesis; this, along with excessive connective tissue production, affects epidermal keratinocytes, and thereby contributes to the changed quality of skin barrier. The objective of this article was to study the objective measurement of the skin barrier quality in LS with transepidermal water loss (TEWL) meter.

[A Case of Delayed Dia-gnosis of Acral Lentiginous Melanoma].

Background: Melanoma is a malignant skin disease. The tumor development is caused by an uncontrollable proliferation of melanocytes. The most common occurrence is on the skin, but melanoma may also develop on the mucous membrane, meninges, and eyes.

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.

[Squamous cell carcinoma in localized scleroderma].

We present a case of a young 26-year-old woman, who has been suffering from localised scleroderma (morphea) for 15 years. Recently, a lesion on the dorsum of her right foot ulcerated. Based on a CT scan and X-ray a diagnosis of ulcerative osteomyellitis was established.

Pemphigus. S2 Guideline for diagnosis and treatment--guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV).

Background: Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, the prognosis of pemphigus was almost fatal. Due to its rarity, only few prospective controlled therapeutic trials are available.

[Mycobacteria at extrapulmonary sites].

Two cases of mycobacterial infections are presented - one of rare hepatic tuberculosis and second of cutaneous mycobacteriosis caused by Mycobacterium chelonae. The aim of the second report is to point to nontuberculous, atypical, otherwise potentially pathogenic mycobacteria. These mycobacteria may cause diseases of various localization and severity in immunocompromised patients.

[Skin adverse effects of amiodarone].

Background: Amiodarone has belonged to frequently used antiarrhythmic in the treatment of supraventricular and ventricular tachyarrhytmias since the sixties of the twentieth century. Amiodarone is a chemically iodinated benzofuran derivative with mono-N-desethylamiodarone as its major metabolite.

Objective: This review is focused on numerous adverse reactions.

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.

Anti-basement membrane zone antibodies in elderly patients with pruritic disorders and diabetes mellitus.

Anti-basement membrane zone (anti-BMZ) antibodies are detectable in a low percentage of elderly subjects without clinical signs of bullous pemphigoid (BP). BP may initially mimic other pruritic dermatoses and may be more common in patients with diabetes mellitus (DM), since DM is frequently associated with pruritic disorders. The aim of the present study was to analyse a possible association of BP and DM and to detect subclinical BP among elderly patients with pruritic dermatoses.

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.

Granuloma faciale successfully treated with topical tacrolimus: a case report.

Granuloma eosinophilicum faciale (GF) is a rare chronic inflammatory disorder of unknown etiology. Although the condition is benign, its treatment is often unsatisfactory. We describe a case of a 60-year-old man with GF resistant to therapy with topical corticosteroids and liquid nitrogen.