Search Results (986)

Background: Behçet-like syndrome (BLS) refers to the presence of Behçet’s disease (BD) features occurring in association with distinct clinical–pathological conditions such as inborn errors of immunity, myeloproliferative disorders, infections, or drug exposure. BLS may differ clinically from BD and is increasingly recognized as a separate entity. Distinguishing BLS from primary BD is essential for appropriate management, and studying BLS may provide insights into BD pathogenesis. Objectives: To summarize clinical features, treatments, and genetic abnormalities reported in BLS, we reviewed all published cases up to January 2024. Methods: A systematic search of PubMed, Scopus, and Embase was performed using the terms “Behçet-like syndrome”, “Behçet-like disease”, and “Pseudo-Behçet disease”. We included English-language reports of patients > 12 years old with a defined underlying etiology and Behçet-like manifestations, defined by ≥2 ICBD criteria and/or gastrointestinal involvement, mucosal ulcers, thrombosis, or non-recurrent disease. Epidemiological, clinical, laboratory, histological, and treatment data were extracted and analyzed descriptively. Results: Of 679 publications, 53 met inclusion criteria, comprising 100 patients with BLS. The median age was 44 years (IQR 22–52), with a female predominance (1:2). Fifty-three percent were from non-European countries. A genetic disorder was identified in 70% of cases, while HLA-B51 was present in 10%. Frequent manifestations included skin lesions (68%), fever (56%), intestinal involvement (43%), and joint symptoms (43%). Treatments included glucocorticoids (65%), conventional DMARDs (32%), and biologics (22%), mainly anti-TNF agents. Antiviral/antibiotic therapy was used in 9% and chemotherapy in 15%. Two patients with trisomy-8 MDS underwent allogeneic stem cell transplantation. Conclusions: Diverse conditions—including monogenic diseases, immune defects, myeloproliferative disorders, infections, and drug-related reactions—can produce Behçet-like features. Our findings highlight differences in clinical expression and treatment response across BLS etiologies. Recognizing BLS is essential for appropriate management and may contribute to a deeper understanding of BD pathogenesis and future targeted therapies. Full article

Hard-to-heal wounds remain a significant challenge for healthcare professionals, particularly in aging populations. Although most chronic wounds are associated with diabetes or chronic venous insufficiency, rare etiologies should also be considered. One such cause is envenomation by Phoneutria spp. (native to South America, rare in Europe). Their venom contains potent neurotoxins. While systemic manifestations are more commonly reported, localized necrotic skin lesions may also occur. This case report presents a rare chronic wound following a suspected Phoneutria spider bite and highlights the importance of an individualized, multimodal treatment approach. A 61-year-old male patient with a progressive thigh wound following a spider bite sustained during work. Despite initial self-treatment and pharmacotherapy the wound deteriorated. The patient was admitted to the authors’ facility, where surgical treatment included necrosectomy and a sandwich graft using an acellular dermal matrix combined with a split-thickness skin graft. Adjunctive therapies included negative pressure wound therapy and hyperbaric oxygen therapy. After discharge, outpatient wound care was continued. Treatment was monitored with photographic documentation and serial microperfusion measurements. Complete wound closure was achieved after 4 months of specialized therapy. Management of chronic wounds requires a multidisciplinary and individualized approach with surgical intervention, advanced wound care and specialized outpatient follow-up. Full article

Geleophysic dysplasia (GD) is a rare genetic skeletal disorder belonging to the acromelic group, characterized by short stature, distinctive facial features, thickened skin, and progressive cardiac involvement. We report a case of a 3-year-old boy with GD caused by a heterozygous c.5198G>A variant in the FBN1 gene, presenting with ocular abnormalities. The patient demonstrated coarse facial features, short hands and feet, and a history of mitral valve stenosis requiring mechanical valve replacement. He was referred to the ophthalmology department for evaluation of left eye strabismus and elevated intraocular pressure. Fundus examination revealed a pink optic disc with blurred margins, slightly elevated above the retinal plane, absent foveal reflex, and tortuous vessels, consistent with optic disc drusen on ocular ultrasonography. Photopic negative response (PhNR) testing showed markedly reduced amplitudes in both eyes, indicating retinal ganglion cell dysfunction. Pattern VEP revealed normal P100 latencies in both eyes, with a 30% reduction in amplitude in the left eye, likely related to poorer fixation. This case highlights optic disc drusen and retinal ganglion cell dysfunction as potential ocular manifestations of geleophysic dysplasia, emphasizing the need for comprehensive ophthalmologic evaluation in affected patients. Full article

Nontuberculous Mycobacteria (NTM), often referred to as environmental or atypical mycobacteria, are opportunistic pathogens phylogenetically as well as clinically distinct from both the Mycobacterium tuberculosis complex and Mycobacterium leprae. In the pediatric age group, NTM disease manifests with a diverse range of clinical phenotypes. Cervicofacial lymphadenitis stands out as the most common presentation among children who are immunocompetent. Conversely, skin and soft tissue infections, pulmonary disease and disseminated infections constitute less prevalent, yet clinically important, disease forms. Accurate identification is paramount, as differentiating NTM infections from tuberculosis (TB) remains challenging based solely on clinical symptoms, initial laboratory analyses, or standard radiological findings. This distinction is critical because treatment protocols for NTM infections differ substantially from those for tuberculosis. This narrative review offers a comprehensive and up-to-date summary of NTM infections in children. It examines the spectrum of clinical presentations and their prevalence, addresses the complexities of diagnosis and therapy, and underscores the importance of differential diagnosis against tuberculosis. Furthermore, we explore current diagnostic strategies, available therapeutic options, and the link between specific clinical syndromes and tailored management, pointing out existing knowledge gaps and suggesting priorities for future research. The absence of rapid, species-specific diagnostic tools often results in delayed initiation of targeted treatment, while overlapping clinical features with TB can lead to misdiagnosis. Therapeutic management is complicated by the necessity for prolonged drug courses, frequent occurrences of drug intolerance, limited availability of child-appropriate formulations, and the rising tide of antimicrobial resistance. Successfully tackling these issues demands enhanced surveillance, precise species-level identification, the creation of child-friendly drug formats, and the development of evidence-based treatment guidelines specifically designed for the pediatric population. Full article

Background: Antimicrobial resistance is a major global challenge that is exacerbated by extensive antibiotic use in livestock farming. Identifying effective alternatives to widely used human antibiotics in animal production is vital to safeguard vital human medicines and ensure sustainable food systems. Here we describe studies identifying nitroxoline (NTX) as a promising antimicrobial candidate for use in poultry production. Methods: The antibacterial activity and resistance potential of NTX were assessed in vitro. In vivo studies in chickens evaluated tolerance, therapeutic efficacy in Salmonella-infected birds, pharmacokinetics, tissue residue depletion, growth performance, and effects on caecal microbiota. NTX was administered in-feed at different dose levels. Pharmacokinetic parameters and withdrawal periods were determined, and caecal microbiota composition was analysed using ribosomal RNA 16S sequencing. Results: NTX exhibits potent broad-spectrum antibacterial activity in vitro and low levels of resistance. NTX is well-tolerated in chickens at 500 mg/kg in-feed for 7 days and substantially reduces liver bacterial loads at 100 mg/kg in Salmonella-infected chickens. Pharmacokinetic and residue analyses reveal NTX manifests rapid absorption and distribution, high oral bioavailability (86%), and efficient tissue clearance with a 17-day withdrawal period required for skin-plus-fat clearance. NTX supplementation is associated with increased weight gain and improved feed efficiency compared to the control group, with performance comparable to chlortetracycline. Microbiota analysis indicates modulation of caecal bacterial communities, including increased Faecalibacterium and Lactobacillus. Conclusions: These results indicate that NTX is a viable alternative to important human antibiotics widely deployed in poultry production, offering a potential approach to minimise antimicrobial resistance whilst maintaining animal health and food biosafety. Full article

Psoriasis is a common inflammatory skin disease with chronic manifestation in which the role of neutrophil extracellular traps (NETs) and alarmins are increasingly recognized as contributors to systemic and cutaneous inflammation. However, the interaction between alarmins and NET-driven immune responses remains poorly defined. The main aim of this study is to define the role of target alarmins (i.e., IL-33 and TSLP) in NETs induction and its subsequent impact on oxidative stress and inflammation in the peripheral blood. In the present study, we recruited active psoriasis patients (n = 56) and control (n = 56) subjects. The frequency of circulating neutrophils, the levels of NET-associated markers (MPO (myeloperoxidase)–DNA complex, CitH3 (citrullinated histone H3), PAD4 (peptidyl arginine deiminase4), NADPH oxidase, and NE (neutrophil elastase)), and alarmin transcripts (IL (interleukin)-33, TSLP (thymic stromal lymphopoietin), S100A7, S100B, HSP (heat shock protein) 60/70 were quantified using flow cytometry, ELISA (Enzyme-linked immunosorbent assay), and qPCR (quantitative polymerase chain reaction), respectively, in each group. The NET formation potential of isolated neutrophils was assessed in the presence or absence of rhIL-33 and rhTSLP by immunocytofluorescence. The effect of rhIL-33- and rhTSLP-primed NETs in augmenting oxidative stress and inflammation was evaluated on peripheral blood mononuclear cells (PBMCs) by ELISA. Significantly higher circulating neutrophils (p < 0.001) and levels of NET-associated markers (i.e., MPO–DNA complex, CitH3, PAD4, NADPH oxidase, and NE) were observed in active psoriasis patients compared to controls. Lesional skin exhibited strong expression of MPO (p < 0.001) compared to normal skin. The alarmins, IL-33 and TSLP, were markedly upregulated in the blood and skin (p < 0.05). The rhIL-33 and rhTSLP treated neutrophils demonstrated enhanced NETosis in patients (p < 0.001). Increased expression of inflammatory cytokines and oxidative stress markers were reported in PBMCs when incubated with rhIL-33- and rhTSLP-primed NETs. Taken together, our investigation demonstrated the novel mechanism wherein the alarmins IL-33 and TSLP exacerbate NET formation that may drive enhanced inflammation and oxidative stress in psoriasis. Full article

The emerging field of dermatogenomics, which examines visible dermatologic phenotypes alongside their polygenic factors, offers insights for early disease recognition and initiation of preventative measures. This review explores key dermatologic manifestations serving as clinical markers of systemic diseases, emphasizing cardiovascular, autoimmune, neuropsychiatric, metabolic/endocrine, and cancer-related conditions. Importantly, the pathogenesis of certain skin conditions including psoriasis, atopic dermatitis, vitiligo, and hidradenitis suppurativa is linked to systemic disease through shared genetic and epigenetic mechanisms. The diagnostic markers for these integumentary diseases are discussed alongside their shared mechanisms to systemic diseases, highlighting the clinical manifestation typically seen in primary care settings. This narrative review integrates dermatology with genomics, primary care, preventative care, public health, and internal medicine perspectives, underscoring the importance of an interdisciplinary and collaborative approach to patient care. Lastly, this review advocates for standardized dermatogenomic screening thresholds, inclusivity and expansion of genomic datasets, and the leverage of artificial intelligence and multi-omic technologies in preventative healthcare. Full article

Background/Objectives: Acne is a common skin condition that is characterized by the manifestation of comedones, erythematous papules, pustules, and nodules over follicular areas. A huge contributing factor in the pathogenesis is colonization by Cutibacterium acnes (C. acnes) (formerly Propionibacterium acnes). Conventional treatments for acne range from topical to systemic agents with variable side effects and safety profiles. Adherence to prescribed treatments for acne is a huge challenge. Method: A quantitative cross-sectional study was conducted among 198 patients with dermatologist-confirmed acne vulgaris at King Abdulaziz University Hospital, Jeddah. Eligible participants had received topical and/or systemic treatment for at least one month. Exclusion criteria included other acne variants and inflammatory follicular disorders. Data on sociodemographics, medical and treatment history, and clinical characteristics were collected using a structured questionnaire. Treatment adherence was assessed with the validated ECOB scale. Associations between adherence and relevant variables were analyzed using Chi-squared and Mann–Whitney tests in SPSS v26, with significance set at p ≤ 0.05. Results: Non-adherence to anti-acne medications was 50.5% and was significantly associated with experiencing side effects, particularly skin dryness, and with moderate acne severity and topical treatment (p ≤ 0.05). No significant associations were found between adherence and demographic or medical history variables. Conclusions: Adherence to acne treatment remains a significant challenge for many patients. Improving patient education, addressing concerns about side effects, and providing practical support may help patients follow their prescribed therapies more consistently. Incorporating tools like the ECOB questionnaire into routine dermatology visits can support ongoing assessment and better management of treatment adherence. Full article

Background: Vogt–Koyanagi–Harada (VKH) is a multisystem autoimmune disease that ophthalmologists often encounter first. The condition is caused by an immune response against tyrosinase-related proteins in pigment cells (melanocytes) of the uvea, inner ear, meninges, and skin, and the process may be triggered by genetic and environmental factors. Although much is known about the disease, establishing an accurate and timely diagnosis still requires a multidisciplinary team and strong clinical expertise. Treatment demands early and aggressive anti-inflammatory therapy with corticosteroids, often prolonged and combined with immunosuppressive or biological agents. Aim: The present article aims to present three unique cases of patients with VKH syndrome, diagnosed and monitored by Ophthalmologists using standard imaging techniques over the course of five years, to demonstrate the unusual manifestations of the already rare syndrome and to improve the general knowledge of the disease among Ophthalmology specialists. Methods: Three different patients with various subjective symptoms and unique clinical signs went through observation in University Specialized Eye Hospital for Active Treatment—Varna. Results: The three clinical cases presented diagnostic challenges, the key role of imaging studies and the importance of thorough medical history taking. Conclusions: The prognosis in VKH is variable—timely diagnosis and treatment are essential to reduce the risk of recurrence and chronic progression of the disease. Full article

Background/Objectives: The skin is an important indicator of overall health, and its relationship with insulin resistance (IR) and metabolic syndrome (MetS) has garnered increasing attention. This review explores the connection between glucose dysregulation and various dermatological conditions, aiming to highlight integrative approaches for management. Methods: A comprehensive literature search was conducted in June and July 2024 across PubMed, Google Scholar, and Embase. Peer-reviewed studies on glucose dysregulation in dermatology were identified using terms such as “insulin,” “metabolic syndrome,” and “dermatological manifestations.” Relevant studies were selected based on their contributions to understanding these relationships. Results: The review identified significant associations between glucose dysregulation, MetS, and conditions such as psoriasis, acne, acanthosis nigricans, seborrheic dermatitis, and hidradenitis suppurativa. Key findings indicated that elevated insulin levels and inflammatory markers correlate with the severity of these skin disorders. Notably, dietary interventions and probiotics show potential in modulating inflammation and improving metabolic health. Conclusions: There is a clear link between glucose dysregulation and several dermatological conditions, underscoring the importance of a holistic treatment approach. By addressing glucose control and incorporating lifestyle modifications, clinicians can improve patient outcomes and mitigate the complications associated with IR and MetS. Further research is essential to refine these integrative strategies and assess their effectiveness in clinical practice. Full article

Aeromycoflora present in the library environment is known to play a significant role in triggering allergies and contributing to the deterioration of both cellulosic and non-cellulosic materials within the intramural setting of the Midnapore College Library. Fungal spores not only accelerate the aging and degradation of books but also pose considerable health risks to students, library visitors, and staff. In total, 480 fungal colonies belonging to 15 genera and 28 species were recorded using the culture plate exposure method. The predominant taxa included Aspergillus/Penicillium, Alternaria alternata, Alternaria solani, Cladosporium cladosporioides, Curvularia lunata, Penicillium oxalicum, Epicoccum sp., Fusarium solanii, Fusarium oxysporum, Periconia sp., Rhizopus sp., and other Penicillium species. Many of these fungi are well-documented allergens and have been reported to cause adverse health manifestations—such as respiratory discomfort and skin irritation—among students, teaching staff, and book handlers exposed to airborne mycobiota. The present study aimed to investigate the aeromycological diversity within the Midnapore College Library and to conduct immuno-clinical assessments to identify specific serum IgE using both in vivo and in vitro diagnostic techniques. Individuals frequently visiting the library reported symptoms including eye irritation, headaches, itchy skin, sore throat, and severe asthma. Spearman’s rank correlation analysis revealed a significant association between total and dominant spore concentrations and the health status of affected individuals. Clinico-immunological evaluations confirmed the allergenicity of Aspergillus fumigatus, with 39.5% of atopic individuals showing positive reactions in skin prick tests (SPT). Additionally, three novel sero-reactive proteins were identified, offering valuable insights for local clinicians in diagnosing and managing fungal-induced allergic conditions. Full article

Background and Clinical Significance: Cutaneous lupus erythematosus (CLE) is an autoimmune condition characterized by a wide range of cutaneous manifestations, classified into three major subtypes—chronic (CCLE), subacute (SCLE), and acute (ACLE)—based on clinical morphology and lesion duration. Discoid lupus erythematosus (DLE), the most common form of CCLE, predominantly affects sun-exposed areas and presents as erythematous macules that progress to well-demarcated, disc-shaped plaques. If left untreated, DLE may lead to scarring and permanent alopecia. Diagnosis is primarily clinical, with skin biopsy performed when indicated. Management includes photoprotection and topical corticosteroids, with systemic immunosuppressive therapy reserved for severe cases. Case Presentation: We report a case of a 38-year-old female patient presenting with confluent lesions with indurated borders and multiple pustules, initially raising suspicion of cutaneous infection. A broad differential diagnosis was considered, including fungal and bacterial infections, demodicosis, and cutaneous tuberculosis, all of which were excluded through comprehensive clinical and laboratory investigations. Ultimately, DLE was diagnosed based on serologic and histopathologic findings. During the course of immunosuppressive therapy, her condition deteriorated, and she developed pulmonary tuberculosis. Conclusions: The presented case underlines the rarity and broad differential diagnosis of DLE as well as the possibility of complications. Full article

Sporothrix schenckii is a thermodimorphic fungus and one of the main etiological agents of sporotrichosis, a globally distributed subcutaneous mycosis that primarily affects the skin, subcutaneous tissue, and lymphatic system. Historically regarded as the classical species within the Sporothrix pathogenic clade, S. schenckii remains a clinically relevant pathogen and an important biological model for studying fungal dimorphism, virulence, and host–pathogen interactions. Major virulence factors include melanin production, thermotolerance, hydrolytic enzymes, and adhesins, all of which contribute to its survival and dissemination within the host. Clinically, S. schenckii causes a broad spectrum of manifestations ranging from fixed and lymphocutaneous cutaneous forms to disseminated and extracutaneous infections, particularly in immunocompromised individuals. This species exhibits a cosmopolitan distribution with endemic foci in the Americas, Asia, and Africa, and can be transmitted through both sapronotic and zoonotic routes. Diagnosis relies on fungal isolation, molecular identification, and histopathological examination, whereas treatment mainly involves itraconazole, potassium iodide, and amphotericin B for severe cases. This review integrates current knowledge on the biology, virulence, immune response, epidemiology, and treatment of S. schenckii, providing an updated overview of its significance as a medically important fungal pathogen with global relevance. Full article

Introduction: Toxic Epidermal Necrolysis (TEN) and Steven–Johnson Syndrome (SJS) are rare yet dangerous dermatological emergencies presenting as necrosis of the skin and mucous membranes due to an immune reaction which may be associated with the use of pharmaceuticals—predominantly non-steroidal anti-inflammatory drugs (NSAIDs), antibiotics, and antiretroviral drugs. During the postpartum period, women are administered numerous pharmaceuticals, including NSAIDs, analgesics, and antibiotics, due to pain and their susceptibility to infections, exposing them to potential adverse effects including allergies and immune reactions. Case Report and Review: The case reported here is a rare description of a patient in the early postpartum phase who presented with epidermal necrolysis whilst remaining hospitalized after a cesarean delivery. The multidisciplinary approach, early diagnosis, and treatment ensured the patient’s full recovery. Intravenous immunoglobulin treatment resulted in a rapid therapeutic effect. This literature review offers an insight into the epidemiology, diagnostic process, and treatment of this infrequent dermatological syndrome during the postpartum period. Results: Antibiotic treatment is a common culprit of TEN in this population; hence, clinicians should remain vigilant during antibiotic administration. Differential diagnosis with toxic shock syndrome is crucial, as TEN and SJS symptoms may mimic sepsis, which is a more common diagnosis in the postpartum period. Conclusions: The condition of the skin during the postpartum period should be closely monitored, as some systemic diseases may manifest abruptly as profound, postpartum hormonal changes affect the immunological response. Upon the discovery of suspicious skin lesions concomitant with systemic symptoms, an immediate multidisciplinary approach involving obstetricians and dermatologists is key to a rapid diagnosis and treatment to avoid maternal mortality. Full article

Allergen sensitization profiles are increasingly affected by environmental and climate changes. This study exemplifies fundamental differences in molecular IgE sensitization profiles in two nearby regions in Argentina with different climatic conditions (La Plata and Bahía Blanca). A cross-sectional study was conducted involving 155 patients with allergic symptoms from La Plata and Bahía Blanca (34.0 ± 11.2 years, female/male: 83/72). Serum samples were analyzed for IgE reactivity using a chip containing 101 micro-arrayed allergen molecules. Statistical analyses were performed to compare allergen-specific IgE levels, sensitization prevalences and reported symptoms. Patients from La Plata—with subtropical weather—showed a higher prevalence of IgE reactivity to house dust mite (HDM) allergens (Der p 23: 74%; Der p 1: 53% and Der p 2: 56%) and more frequently reported asthma (AS) symptoms (40% vs. 24%) than patients from Bahía Blanca. In contrast, patients from Bahía Blanca, with dry and windy weather, exhibited higher sensitization rates to pollen allergens, particularly Phl p 1 (49%) and Ole e 1 (22%) as well as to Alternaria alternata (Alt a 1, 35%) and reported a significantly higher prevalence of skin manifestations (54% vs. 31%) than those from La Plata. Cat allergen Fel d 1 was an equally important sensitizer in both regions (La Plata 30% and Bahía Blanca 37%). Sensitization to class 1 food allergens was rare in both groups (1–8%), including non-specific lipid transfer proteins (peanut Ara h 9 and peach Pru p 3) but IgE sensitizations to genuine peanut allergens were almost absent. Important regional differences in allergen sensitization profiles were observed between two geographically close regions with different climatic conditions. Our findings underscore the relevance of region-specific allergen profiling and highlight the clinical utility of molecular allergy diagnosis for a more precise allergen identification and improved management of allergic diseases. Full article