Dermatomyositis: a comprehensive review of clinical manifestations, serological features, and therapeutic approaches.

Dermatomyositis (DM) is an autoimmune disorder, which belongs to a group of rare autoimmune dermatoses characterized by different skin features and variable muscle involvement. We recognize four main variants of DM: classic DM, clinically amyopathic DM, paraneoplastic DM, and juvenile DM. Clinically, patients show several skin features, but heliotrope rash, and violaceous papules located at the interphalangeal or metacarpophalangeal joints (Gottron's papules) are the most frequently observed. Together with skin features, patients show muscle involvement, most commonly with symmetrical weakness of the proximal muscles. DM belongs to the facultative paraneoplastic dermatoses and a wide range of solid or hematologic malignancies can be detected in DM patients. Serologically, a wide range of autoantibodies can be detected in patients with DM. Indeed, distinct serotypes can be related to specific phenotypes with specific clinical features, carrying a different risk for systemic involvement and for malignancies. Systemic corticosteroids are still considered the first-line approach, but several steroid-sparing agents, such as methotrexate, azathioprine or mycophenolate mofetil, have been reported as effective in treating DM. Furthermore, new class of drugs, such as monoclonal antibodies, purified immunoglobulins or Janus kinase inhibitors are becoming more relevant in the clinical practice or are currently under investigation. In this work, we aim to offer a clinical overview of the diagnostic workout, the characteristics of DM variants, the role of autoantibodies in DM, and the management of this life-threatening systemic disorder.

Autoimmune skin disorders and SARS-CoV-2 vaccination - a meta-analysis.

BACKGROUND AND OBJECTIVES: The coronavirus SARS-CoV-2, which is the cause of COVID-19 disease in infected patients, has led to an ongoing worldwide pandemic. Although SARS-CoV-2 vaccination had a dramatic positive effect on the course of COVID-19, there has been increasing evidence of adverse effects after SARS-CoV-2 vaccination. This meta-analysis highlights the association between SARS-CoV-2 vaccination and de novo induction or aggravation of inflammatory and autoimmune skin diseases. MATERIAL AND METHODS: A systematic meta-analysis of the literature on new onset or worsening of inflammatory and autoimmune diseases after SARS-CoV-2 vaccination was performed according to the PRISMA guidelines. The search strategy included following terms: "COVID-19/SARS-CoV-2 vaccine bullous pemphigoid/pemphigus vulgaris/systemic lupus erythematosus/dermatomyositis/lichen planus/leukocytoclastic vasculitis." Moreover, we describe representative cases from our dermatology department. RESULTS: The database-search in MEDLINE identified 31 publications on bullous pemphigoid, 24 on pemphigus vulgaris, 65 on systemic lupus erythematosus, nine on dermatomyositis, 30 on lichen planus, and 37 on leukocytoclastic vasculitis until June 30th, 2022. Severity and response to treatment varied among the described cases. CONCLUSIONS: Our meta-analysis highlights a link between SARS-CoV-2 vaccination and new onset or worsening of inflammatory and autoimmune skin diseases. Moreover, the extent of disease exacerbation has been exemplified by cases from our dermatological department.

Case report: Detection of anti-bullous pemphigoid antigen 180 antibodies in a patient with Behçet's disease.

Behçet's disease (BD) is a systemic inflammatory disease of unknown etiology. BD is characterized by relapsing oral and genital ulcers, several different cutaneous features, relapsing bilateral uveitis, and involvement of internal organs, showing vascular, gastrointestinal, and neurological manifestations. Serologically, BD is not characterized by disease-specific autoantibodies. In fact, only laboratory markers of inflammation, such as C-reactive protein, may be increased in association with increased disease activity. Bullous pemphigoid (BP) is an autoimmune disease characterized mainly by tense blisters and urticaria-like plaques on the skin. In addition, BP can involve oral mucosa in up to 20% of patients. Patients with BP show serum IgG autoantibodies against BP antigen 180 (BP180) and/or BP antigen 230 (BP230). Tissue-bound autoantibodies can be visualized as linear IgG staining along the basement membrane by direct immunofluorescence microscopy. In this report, we first described a young patient with BD who showed IgG autoantibodies against BP180 without developing blisters or urticaria-like plaques.

[Bullous autoimmune dermatoses of the mucous membranes]. / Bullöse Autoimmundermatosen der Schleimhaut.

Autoimmune bullous diseases (AIBD) comprise a group of organ-specific autoimmune diseases which are characterised by the production of autoantibodies against adhesion molecules and structural proteins of skin and mucosae. Depending on the target protein, AIBD are classified into intraepidermal (pemphigus group) and subepidermal (pemphigoid group, epidermolysis bullosa acquisita, dermatitis herpetiformis) blistering disorders. Depending on the clinical entity, patients can develop blisters, pustules, erosions, and erythema on the skin and mucosae.

Milk Polysialic Acid Levels Rapidly Decrease in Line with the N-Acetylneuraminic Acid Concentrations during Early Lactation in Dairy Cows.

Sialylated milk oligosaccharides and glycoconjugates have several positive effects on the mucosal barrier, the gut microbiome, and an effective immune system. For this reason, they are important biomolecules for mammary gland health and optimal development of offspring. In milk, the major sialic acid, N-acetylneuraminic acid (Neu5Ac), can be attached as monosialyl-residues or as polymers. To investigate the sialylation processes during lactation of German Holstein cows, we analyzed udder tissue in addition to milk at different time points of lactation. The analysis of the milk samples revealed that both the levels of Neu5Ac and its polymer, polysialic acid (polySia), rapidly decreased during the first three days of lactation, and a high interindividual variance was observed. In mature milk, however, the sialylation status remains relatively constant. The results indicate that mammary gland epithelial cells are one source for milk polySia, since immunohistochemistry of udder tissue exhibited strong polySia staining in these cells. Furthermore, both polysialyltransferases, ST8SiaII and ST8SiaIV, are expressed. Based on known functions of monosialyl residues and polySia, we discuss the potential impact of these biomolecules and the consequences of the heterogeneous sialylation status of milk in relation to udder health and offspring health.