bullous diseases

No abstract text is available yet for this article.

BACKGROUND: Fixed drug reaction (FDE) is characterized by the development of well-circumscribed, round, erythematous macules and plaques on cutaneous or mucosal surface following ingestion of the offending drug. AIM AND OBJECTIVES: To study the etiological agents responsible for FDE and to study the clinical patterns of FDE due to different drugs. MATERIALS AND METHODS: It was a hospital-based observational cross-sectional clinical study. The study period was 24 months...

BACKGROUND: The association between psoriasis vulgaris and bullous pemphigoid (BP) remains largely unknown. OBJECTIVES: To investigate whether there is a causal effect between psoriasis vulgaris and BP. METHODS: Two-sample bidirectional Mendelian randomization (MR) analyses were conducted using publicly released genome-wide association studies (GWAS) summary statistics. The GWAS summary statistics for BP were downloaded online from FinnGen Biobank Documentation of the R12 release, which includes 219 BP cases and 218,066 controls...

Background: Descemet Membrane Endothelial Keratoplasty (DMEK) has been widely adopted to treat Fuchs endothelial dystrophy (FED) and Bullous keratopathy (BK). Graft detachment (GD) is one of the common earliest post-operatory complications, and it is usually recovered by Air Rebubbling (ARB). Methods: Retrospectively, we investigated predictive factors related to GD between January 2016 and March 2020, a pre-COVID era, in 72 patients, 72 eyes, and their donors' lamellar characteristics, focusing on donor's cause of death...

Bullous pemphigoid (BP) is a subepidermal blistering skin disease with a complex pathogenesis involving various immune cells. However, the transcriptional features of these cells remain poorly defined. Here, we construct a comprehensive and single-cell resolution atlas of various immune cells within BP skin lesions via integrative single-cell analysis, flow cytometry and multiplex immunohistochemistry. We observed prominent expansion and transcriptional changes in mast cells, macrophages, basophils, and neutrophils within BP lesions...

BACKGROUND: Following the RITUX 3 therapeutic trial, the French national diagnosis and care protocol (NDCP) for the treatment of pemphigus was updated in 2018. The updated protocol recommends initial treatment with rituximab (RTX) followed by maintenance therapy at 12 and 18 months, and potentially at 6 months where there are risk factors for early relapse. We evaluated these recommendations regarding the management of our own patients. PATIENTS AND METHODS: Our single-center retrospective study included all patients with pemphigus diagnosed between 01/2015 and 10/2020 and receiving at least one initial infusion of RTX...

The Klotho loss-of-function mutation is known to cause accelerated senescence in many organs, but its effects on the cornea have not been published. The present study aims to investigate the effects of the Klotho null mutation on cornea degeneration and to characterize the pathological features. Mouse corneas of Klotho homozygous, heterozygous, and wild-type mice at 8 weeks of age for both genders were subject to pathological and immunohistological examinations. The results show an irregular topography on the corneal surface with a Klotho null mutation...

Claudin-4 is a key component of tight junctions, which play an important role in the formation of the epidermal barrier by forming a circumferential network in the granular layer that serves as a gatekeeper of the paracellular pathway. The aim of this study is to illustrate claudin-4 immunohistochemical staining patterns of different blistering disorders. We collected 35 cases, including two Hailey-Hailey disease, one Darier disease, three Grover disease, one acantholytic acanthoma, two warty dyskeratoma, 11 pemphigus vulgaris (PV) including six mucosal PV, and two pemphigus foliaceus...

We present a case of bilateral giant bullous emphysema (GBE) with rapidly progressive dyspnea. The dyspnea was thought to be due to tension bullae caused by the check valve mechanism in COVID-19 bronchitis. Multiple nodules were also detected on both sides of the lung. As the patient had poor pulmonary reserve for surgical bullectomy, we first performed percutaneous intracavitary drainage. Prior to this procedure, we placed a chest tube in the thoracic cavity to avoid tension pneumothorax. As a result, the patient's remaining lung expanded and respiratory status improved, allowing him to undergo surgical bullectomy...

Aside from rheumatoid arthritis, methotrexate is also used to treat cancer, psoriasis, and other diseases. Side effects with methotrexate are possible, as they are with any medication. This drug is extremely potent and has the potential to produce serious adverse effects. Those who use this medication need to be tracked often. We provide a case of a patient with psoriasis vulgaris who died due to methotrexate administration without proper dosage verification. A female patient in her forties had a history of psoriasis vulgaris of the lower limbs...

Background Chronic autoimmune bullous diseases have been associated with major depression in previous studies. This has been attributed to inflammatory cytokines, chronic pain, and the chronicity and debilitating nature of the disease. As no similar studies have been conducted in our setting, we aimed to determine the prevalence and severity of clinically undiagnosed depression in patients with autoimmune bullous diseases. Methodology We performed a cross-sectional study among outpatients managed in a bullous disease clinic at Inkosi Albert Luthuli Central Hospital, a quaternary provincial hospital in Durban, South Africa...

Dipeptidyl peptidase-4 (DPP-4) inhibitors are a class of drugs that enhance the incretin-insulin pathway and offer effective glycemic control in type 2 diabetes mellitus. However, these drugs may be associated with various dermatological side effects, ranging from mild to severe. This review article summarizes the current literature on the dermatological side effects of DPP-4 inhibitors, including bullous pemphigoid, severe cutaneous adverse drug reactions, fixed drug eruptions, and other mucocutaneous reactions...

Associations between HLA genotype and disease susceptibility encompass almost all the classic HLA loci. The level of typing resolution enabling a correct identification of an HLA disease susceptibility gene depends on the disease itself and/or on the accumulated knowledge about the molecular involvement of the HLA allele(s) engaged. Therefore, the application of Next Generation Sequencing technologies to HLA disease association, which would improve typing resolution, could prove useful to better understand disease severity...

No abstract text is available yet for this article.

BACKGROUND: Bullous pemphigoid (BP) is an antibody-mediated blistering disease predominantly affecting the elderly. The pathogenesis involves both complement-dependent and complement-independent mechanisms. The therapeutic potential of targeting complement-independent mechanism has not yet been determined. The mainstay of treatment, corticosteroid, has many side effects, indicating the needs of better treatments. OBJECTIVE: We tempted to establish an in vitro model of BP which resembles complement-independent mechanism and to examine the therapeutic potential of a novel anti-inflammatory agent, diacerein...

No abstract text is available yet for this article.

Pemphigus vulgaris is a rare autoimmune bullous disease characterized by blistering of the skin and mucosa owing to the presence of autoantibodies against the desmosome proteins desmoglein 3 and occasionally in conjunction with desmoglein 1. Fundamental research into the pathogenesis of PV has revolutionized its treatment and outcome with rituximab, a B-cell-depleting therapy. The critical contribution of B cells to the pathogenesis of pemphigus is well accepted. However, the exact pathomechanism, mechanisms of onset, disease course, and relapse remain unclear...

Staphylococcus aureus is a leading cause of skin and soft tissue infections. Colonization by this bacterium is increased in individuals with chronic cutaneous diseases such as atopic dermatitis, psoriasis, and bullous pemphigoid. The greater abundance of S. aureus on the skin of subjects with atopic dermatitis in particular has been linked to recurrent cutaneous infections. The primary cell type of the epidermal layer of the skin is the keratinocyte, and it is thought that S. aureus internalized in keratinocytes associates with an increased incidence of skin infections...

BACKGROUND: Autoimmune blistering disorders (ABDs) might elevate cardiovascular risk, but studies are lacking. OBJECTIVE: To examine if ABDs elevate the risk of atherosclerotic cardiovascular disease (ASCVD), heart failure, arrhythmia, venous thromboembolism, and cardiovascular death. METHODS: A population-based cohort of Danish patients with ABD (≥18 years of age) diagnosed during 1996-2021 (n=3,322) was compared with an age and sex-matched comparison cohort from the general population (n=33,195)...

Bullous pemphigoid (BP), an autoimmune subepidermal blistering disease, shows tense blisters associated with urticarial erythema. Tissue-bound Immunoglobulin G (IgG) at the basement membrane zone (BMZ) detected by direct immunofluorescence (DIF) is strong evidence for a diagnosis of BP. The sensitivity of DIF is higher in complement component 3 (C3) than in IgG, but the reason for this different sensitivity is not fully understood. In this study, we performed several ex vivo studies to investigate the possible mechanism of IgG negativity and C3 positivity at the BMZ by DIF in some BP cases...