immunobullous

OBJECTIVE: To determine the concordance among clinical, histopathological and immunofluorescence as diagnostic methods for intraepidermal immunobullous disorders. METHODS: The prospective cross-sectional study was conducted at the Institute of Skin Diseases, Karachi, from December 2020 to December 2022, and comprised adult patients of either gender presenting with complaints of bullae, vesicles, pustules and crusts on the skin or mucous membrane. Diagnostic findings of each patient as obtained by clinical assessment, microscopy and direct immunofluorescence were compared...

Myelodysplastic syndrome (MDS) may present with specific skin lesions, such as leukaemia cutis, which is a well known poor prognostic marker of leukaemia with a high risk of acute leukaemic transformation. However, less is known regarding non-specific cutaneous manifestations of MDS including the prevalence, types and their prognostic and therapeutic significance, which we aimed to determine through this systematic review. We searched electronic databases (PubMed, Medline and EMBASE) from inception up to 26 January 2023 for studies reporting cutaneous manifestations of MDS...

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BACKGROUND: There are several methods for the diagnosis of autoimmune bullous disease. Direct immunofluorescent (DIF) testing is an important diagnostic method in the diagnosis of immunobullous disease but requires skilled pathologist, fresh tissue and well-equipped laboratory to perform the procedure. The immunohistochemistry analysis of C4d and C3d is easily compared with other methods. This study was conducted to assess the value of immunohistochemistry (IHC) analysis for expressions of C3d and C4d in the diagnosis of bullous pemphigoid (BP)...

OBJECTIVES: Azathioprine (AZA) is an effective immunosuppressant commonly used for malignancy and immune-mediated disorders. The association between genetic polymorphisms and AZA-induced adverse effects has not been elucidated. Hence this study aimed to evaluate the relationship between single nucleotide polymorphisms of ITPA (C94A) with azathioprine-induced adverse effects. METHODS: A cross-sectional study was performed on 120 patients who were on AZA therapy for immunobullous disorders and inflammatory bowel disease (IBD)...

Autoimmune blistering diseases (AIBD) comprise a heterogeneous group of uncommon disorders of the skin and mucous membranes, characterised by antibodies targeting structural proteins within epithelial tissue and the underlying basement membrane. There can be significant overlap in clinical presentation of these diseases and accurate diagnosis relies on the detection and characterisation of relevant autoantibodies. Immunofluorescence provides the gold-standard diagnostic tool for these diseases, identifying both tissue-bound autoantibodies in biopsy material using direct immunofluorescence and circulating antibodies in serum through indirect immunofluorescence...

Immune checkpoint inhibitors (ICIs) have revolutionized treatment strategies in the field of oncology. Their favourable outcomes in terms of efficacy and side-effect profile can be thwarted by the development of immune-related adverse events (irAEs). Cutaneous irAEs are relatively common in patients undergoing immunotherapy and include common inflammatory dermatoses (e.g. eczematous, psoriasiform and lichenoid phenotypes), maculopapular eruptions, pruritus and immunobullous disorders. Most of these reactions can be managed without ICIs having to be stopped completely; however, there are some life-threatening toxicities that dermatologists and oncologists should be aware of...

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No abstract text is available yet for this article.

Erythroderma is a general term used to describe severe, intense skin inflammation. The condition is also known as exfoliative dermatitis when it is associated with exfoliation. Erythroderma has many causes, such as adverse drug eruption, dermatitis, psoriasis, pityriasis rubra pilaris, immunobullous disease, cutaneous T-cell lymphoma (Sézary syndrome), underlying systemic malignancy, graft versus host disease, and HIV infection. Many medications can cause erythroderma, including antibiotics, antiepileptics, angiotensin-converting enzyme (ACE) inhibitors, and sulfonamides...

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Bullous pemphigoid has a high incidence among dialysis patients. However, whether or not chronic dialysis is an independent risk factor of bullous pemphigoid remains unclear. We aimed to investigate the effect of chronic dialysis on the development of bullous pemphigoid and pemphigus. We performed a retrospective cohort study using records from Taiwan's National Health Insurance Research Database between 2008 and 2019. We identified a dialysis cohort that included patients on chronic hemodialysis and peritoneal dialysis, and the hazard ratios (HRs) for bullous pemphigoid and pemphigus were compared with those of a sex-, age-, and index-matched cohort, then the results were adjusted for various confounding factors...

Background Direct immunofluorescence (DIF) is essential for the diagnosis of sub-epidermal immunobullous diseases (SIBD). Bullous pemphigoid (BP), a sub-epidermal immunobullous disease, shows linear IgG and C3 deposition along the dermo-epidermal junction by DIF. However, similar histological and DIF findings are also seen in epidermolysis bullosa acquisita (EBA). High-power examination of antibody deposition by DIF in a "u" or "n" serrated pattern can help differentiate these two entities. Aims/Objectives The aim of this study was to determine the diagnostic accuracy of serration patterns in IgG-mediated sub-epidermal immunobullous disease...

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KEY CLINICAL MESSAGE: There may be a connection between pemphigus vulgaris and nephrotic syndrome, as evidenced by the occurrence of focal segmental glomerulosclerosis in our pemphigus vulgaris patient and reviewing relevant literature. Therefore, if a patient with pemphigus vulgaris presents with bilateral lower extremity edema or proteinuria detected during urinalysis, it could indicate involvement of the kidneys. ABSTRACT: Pemphigus vulgaris is a type of autoimmune blistering disease characterized by the presence of IgG autoantibodies against desmogleins 3 and 1...

UNLABELLED: The COVID-19 pandemic has highlighted a continued need for innovative virtual teaching methods. Chalk talks, or brief illustrated interactive talks, are easily made virtual with an online whiteboard. We evaluated the efficacy of a live virtual chalk talk curriculum for medical students on their dermatology clerkship. A curriculum of one to three 1-h chalk talks was designed on the following topics: papulosquamous diseases, erythroderma, and immunobullous diseases. Talks were delivered monthly via Zoom to dermatology clerkship students...

We present a rare case of linear IgA bullous dermatosis (LABD) in a 72-year-old male associated with the use of azithromycin. LABD presents as subepidermal blisters due to IgA antibodies targeting BPAG2, a component of hemidesmosomes. LABD is a rare diagnosis and may be idiopathic, associated with illness, or medication-induced. The patient experienced a rash five days after completing a course of azithromycin for pneumonia. The diagnosis of LABD was confirmed with a biopsy and direct immunofluorescence...

CONTEXT: Autoimmune blistering (AIBD) disorders affect mucosae, skin, and appendages. Appendageal involvement has not been studied extensively to date. However, they are important as nail changes are commonly encountered during a flare-up of the disease. AIMS: To determine the prevalence and patterns of nail changes in various Immunobullous disorders and to study the relationship between the nail changes and the disease duration and severity. SETTINGS AND DESIGN: A cross-sectional study was conducted at the Department of Dermatology, venerology, and leprosy at a tertiary care center in Mumbai...

Pemphigus vulgaris is a rare autoimmune disorder, characterised by the development of blistering lesions in the skin and mucosal surfaces throughout the body. It is often misdiagnosed or missed completely in many patients, prolonging their suffering for many years, as it has the ability to mimic an array of other skin diseases. Many studies have concluded that there is a strong association between pemphigus vulgaris and psoriasis, though the exact mechanism is not clearly understood. We present the case of a 77-year-old gentleman on long-term treatment for psoriasis with ultraviolet B phototherapy, steroids, and many other topical treatments who eventually went on to develop pemphigus vulgaris...

The differential diagnosis of vesiculobullous lesions can be intimidating to the primary care provider. While some entities such as bullous impetigo may easily be diagnosed clinically if the patient's demographics as well as the lesion morphology and distribution present classically, atypical presentations may require additional laboratory studies for confirmation. We describe a case of bullous impetigo with characteristics that clinically mimicked two rare immunobullous dermatoses. Although extensive diagnostic testing was performed, we recommend an approach for primary care providers to initiate empiric treatment while maintaining awareness of less common immunobullous entities...