Explore the vast range of titles available.

Generalized pustular psoriasis (GPP) is a severe, relapsing, immune-mediated disease characterized by the presence of multiple sterile pustules all over the body. The exact pathomechanisms behind GPP remain elusive, although increased interest in the genetic basis and immunological disturbances have provided some revealing insights into the underlying signaling pathways and their mutual interaction. The genetic background of GPP has been thoroughly investigated over the past few years. The conducted studies have identified genetic variants that predispose to pustular forms of psoriasis. The loss-of-function mutation of the interleukin 36 receptor antagonist gene, along with rare gain-of-function mutations in the gene that encodes the keratinocyte signaling molecule (CARD14), are examples of the uncovered abnormalities. Interleukin 36 (IL-36), along with neutrophils, is now considered a central cytokine in GPP pathogenesis, with IL-36 signaling providing a link between innate and adaptive immune responses. More recently, a new concept of inflammation, caused by a predominantly genetically determined abnormal activation of innate immune response and leading to inflammatory keratinization, has arisen. GPP is currently considered a representative of this novel group of skin conditions, called autoinflammatory keratinization diseases. As no therapeutic agents have been approved for GPP to date in the United States and Europe, the novel anti-IL-36R antibodies are particularly promising and may revolutionize management of the disease.

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease affecting multiple organ systems that runs an unpredictable course and may present with a wide variety of clinical manifestations. Advances in treatment over the last decades, such as use of corticosteroids and conventional immunosuppressive drugs, have improved life expectancy of SLE sufferers. Unfortunately, in many cases effective management of SLE is still related to severe drug-induced toxicity and contributes to organ function deterioration and infective complications, particularly among patients with refractory disease and/or lupus nephritis. Consequently, there is an unmet need for drugs with a better efficacy and safety profile. A range of different biologic agents have been proposed and subjected to clinical trials, particularly dedicated to this subset of patients whose disease is inadequately controlled by conventional treatment regimes. Unfortunately, most of these trials have given unsatisfactory results, with belimumab being the only targeted therapy approved for the treatment of SLE so far. Despite these pitfalls, several novel biologic agents targeting B cells, T cells, or cytokines are constantly being evaluated in clinical trials. It seems that they may enhance the therapeutic efficacy when combined with standard therapies. These efforts raise the hope that novel drugs for patients with refractory SLE may be available in the near future. This article reviews the current biological therapies being tested in the treatment of SLE.

Psoriasis is a common, chronic, inflammatory, immune-mediated skin disease affecting about 2% of the world's population. According to current knowledge, psoriasis is a complex disease that involves various genes and environmental factors, such as stress, injuries, infections and certain medications. The chronic inflammation of psoriasis lesions develops upon epidermal infiltration, activation, and expansion of type 1 and type 17 Th cells. Despite the enormous progress in understanding the mechanisms that cause psoriasis, the target cells and antigens that drive pathogenic T cell responses in psoriatic lesions are still unproven and the autoimmune basis of psoriasis still remains hypothetical. However, since the identification of the Th17 cell subset, the IL-23/Th17 immune axis has been considered a key driver of psoriatic inflammation, which has led to the development of biologic agents that target crucial elements of this pathway. Here we present the current understanding of various aspects in psoriasis pathogenesis.

Pruritus is an important symptom frequently accompanying various inflammatory skin conditions. Some recent data have indicated that it may also be associated with autoimmune connective tissue diseases, including systemic sclerosis and dermatomyositis; however, studies on the prevalence and clinical characteristics of pruritus in CLE are limited. We have performed a multinational, prospective, cross-sectional study in order to assess the prevalence and intensity of pruritus in adult patients suffering from various subtypes of CLE. After developing a questionnaire assessing various aspects of pruritus, we have surveyed 567 patients with cutaneous involvement during the course of LE regarding the presence and intensity of pruritus. Pruritus was present in 425 of all patients (75.0%) and was most frequently reported by subjects with acute CLE (82.1%), followed by chronic CLE (78.8%), subacute CLE (65.9%), and intermittent CLE (55.6%) (p<0.001). Based on the Numerical Rating Scale, the severity of itch was mild, moderate, and severe in 264 (62.1%), 98 (23.1%), and 63 (14.8%) patients, respectively. The highest mean pruritus intensity was reported by subjects with hypertrophic LE ( 5.1 ± 3.0 points) followed by generalized discoid LE ( 3.6 ± 3.0 points), subacute CLE ( 3.0 ± 3.0 points), chilblain LE ( 3.0 ± 1.0 points), localized discoid LE ( 2.6 ± 2.0 points), intermittent CLE ( 2.6 ± 3.0 points), acute CLE ( 2.5 ± 1.2 points), and lupus erythematosus profundus ( 1.9 ± 2.7 points). In conclusion, pruritus is a frequent phenomenon in CLE; however, in most patients it is of mild severity. Further studies are needed to better characterize its clinical characteristics and influence on patients’ well-being.

Rheumatoid neutrophilic dermatitis is a rare extra-articular manifestation of rheumatoid arthritis, both seropositive and seronegative for rheumatoid factor. The condition most often presents as symmetric erythematous papules, nodules, plaques, and urticaria-like lesions in patients with severe, long-lasting rheumatoid arthritis. We report a case of a 65-year-old man with well-controlled rheumatoid arthritis, who developed rheumatoid neutrophilic dermatitis on the right lower leg. The biopsy of skin lesions revealed an intense, neutrophilic dermal infiltrate, microabscesses, and leukocytoclasis without vasculitis. The patient responded well to pulses of intravenous methylprednisolone. We present this patient due to the rarity of the disease and atypical unilateral manifestation involving the flexural surfaces of the lower leg.

Dermatomyositis (DM) is one of the so-called idiopathic inflammatory myopathies (IIM). Dermatomyositis is an autoimmune disorder characterized by the presence of cutaneous lesions and/or symptoms of muscle involvement with the following variants: the “classic” variant of DM, juvenile DM, paraneoplastic DM, drug-induced DM and amyopathic DM. Dynamic discoveries of novel autoantibodies, including anti-TIF1, anti-NXP2, anti-SAE or anti-MDA5, related to certain DM variants, have been described in recent years. It seems that these antibodies will contribute to better recognition of DM and its particular variants, a better risk stratification for predicting internal organ involvement, and to a global improvement of treatment outcome. Corticosteroids remain the mainstay of DM therapy, but new immunomodulatory and immunosuppressive agents, including biologicals, resulted in a significant increase of the survival rate of DM patients. Here, we review the current literature data on DM with special emphasis on new trends in its diagnostics and treatment.

Background/Objectives: Rheumatoid neutrophilic dermatosis (RND) is a rare extra-articular manifestation of rheumatoid arthritis (RA) with variable clinical presentations. Although typically non-blistering, a rare bullous or vesiculobullous subtype has been described, mainly in patients with seropositive and active RA, and may mimic autoimmune blistering diseases. The objective of this review was to systematically summarize the clinical, histopathological, immunopathological, and therapeutic features of vesiculobullous rheumatoid neutrophilic dermatosis. Methods: A systematic literature review was conducted in accordance with the PRISMA 2020 guidelines utilizing the PubMed, MEDLINE, and Google Scholar databases, which were searched through December 2025. Case reports and case series describing vesiculobullous or bullous RND with extractable patient-level data were included. Non-English articles were translated. Demographic, clinical, histopathological, immunopathological, microbiological, and therapeutic data were extracted and analyzed using Statistica 12.0 software. Results: Results were synthesized descriptively due to clinical heterogeneity and limited sample size. Thirty reported cases were identified, of which 28 non-duplicate cases were included. The mean patient age was 60.8 ± 14.9 years, with a female predominance (male-to-female ratio, 1:2.5). Most patients were of Asian descent (67.9%). Bullous or vesicular lesions most frequently involved the lower legs (64.3%), palms and soles (41.7%), and thighs (35.7%). Rheumatoid factor data were available in 67.9% of patients, all indicating high RA activity. Histopathological examination was reported in 71.4% of cases and most commonly demonstrated a predominantly neutrophilic infiltrate, often dense and extending throughout the dermis, with subepidermal blister formation being the most frequent pattern. Direct immunofluorescence, serological testing for autoimmune bullous diseases, and microbiological investigations were predominantly negative. Dapsone and systemic corticosteroids, alone or combined with RA-specific therapies, were the most commonly used treatments. Conclusions: This review represents the most comprehensive synthesis to date focused exclusively on the bullous/vesiculobullous subtype of RND, highlighting key diagnostic features such as neutrophil-predominant histopathology, negative direct immunofluorescence, and favorable response to dapsone.

Introduction. Morphea (or localized scleroderma) is an inflammatory, immune-mediated disease of unknown etiology. It is characterized by excessive collagen deposition that leads to hardening of the dermis, subcutaneous tissues, or both. Morphea is associated with cosmetic and functional impairment, which might affect the patients’ quality of life (QoL). Objective. The aim of the study was to evaluate QoL in patients suffering from morphea. Material and Methods. Sixty-five patients with morphea were recruited into this cross-sectional, prospective parallel study. QoL among adult patients was assessed with the Dermatology Life Quality Index (DLQI) and Euro-QoL-5D questionnaire; patients aged <17 years used the Children’s Dermatology Life Quality Index (CDLQI). The severity of morphea was assessed using the Localized Scleroderma Cutaneous Assessment Tool. The results of QoL and its association with disease severity were compared between patients with various morphea subtypes. Results. The mean DLQI scoring was 3.8±4.1 points and the CDLQI was 2.3±3.0. The mean value of Visual Analogue Scale thermometer (EQ VAS) was 66.9±17.5 points. The disease activity of morphea based on mLoSSI correlated significantly with QoL impairment according to the DLQI (R=0.41, p=0.001). No significant correlation was observed between morphea-induced damage and QoL (p=0.99). Conclusions. Evaluation of QoL in patients with morphea is still challenging due to lack of good assessment tools dedicated specifically for morphea patients. In general, QoL in morphea patients is significantly correlated with the disease activity, but not with disease-induced skin damage.

The global pandemic of coronavirus disease 2019 (COVID-19) caused by the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is emerging. Various cutaneous manifestations have been observed in patients with SARS-CoV-2 infection, yet exacerbations of psoriasis have been reported sporadically. Acrodermatitis continua of Hallopeau (ACH) is an uncommon, sterile pustular dermatosis involving one or more digits. In some rare cases, ACH may evolve into generalized pustular psoriasis (GPP), which is a severe, and potentially life-threatening, form of psoriasis that manifests itself with widespread eruptions of pustules. We describe the first case of a patient in whom ACH abruptly progressed into GPP during COVID-19. A combination of infliximab and acitretin was used allowing swift clinical improvement.

There is evidence that the concomitance of psoriasis and obesity may originate from the interplay between multiple genetic pathways and involve gene–gene interactions. The aim of this study was to compare the genetic background related to obesity among psoriatic patients versus healthy controls by means of a Genome-Wide Association Study (GWAS). A total of 972 psoriatic patients and a total of 5878 healthy donors were enrolled in this study. DNA samples were genotyped for over 500,000 single nucleotide polymorphisms (SNPs) using Infinium CoreExome BeadChips (Illumina, San Diego, CA, USA). Statistical analysis identified eleven signals (p < 1 × 10−5) associated with BMI across the study groups and revealed a varying effect size in each sub-cohort. Seven of the alternative alleles (rs1558902 in the FTO gene, rs696574 in the CALCRL gene, as well as rs10968110, rs4551082, rs4609724, rs9320269, and rs2338833,) are associated with increased BMI among all psoriatic patients and four (rs1556519 in the ITLN2 gene, rs12972098 in the AC003006.7 gene, rs12676670 in the PAG1 gene, and rs1321529) are associated with lower BMI. The results of our study may lead to further insights into the understanding of the pathogenesis of obesity among psoriatic patients.