linear iga bullous dermatosis

No abstract text is available yet for this article.

Autoimmune bullous diseases represent a heterogenous group of disorders caused by autoantibodies against adhesion molecules; the location of the target protein determines the level of cleft formation. The spectrum of ocular lesions in autoimmune bullous diseases can range from mild symptoms to severe involvement with sight impairment and even, in some cases, blindness. In pemphigus vulgaris, the prevalence of ocular involvement has been reported to be between 7% and 26%. The most common clinical sign of ocular pemphigus vulgaris is bilateral conjunctivitis with hyperemia...

BACKGROUND: Pemphigoid diseases are characterized by subepidermal blister formation accompanied by autoantibodies targeting skin component molecules, such as BP180. It is suggested that an epitope-phenotype correlation exists among autoantibodies recognizing BP180. However, it is unclear which regions of BP180 are likely targets for autoantibodies. OBJECTIVE: To elucidate the portions of BP180 where antibodies tend to react under the breakdown of immune tolerance...

Linear IgA bullous dermatosis (LABD) is a rare, idiopathic, or drug-induced vesiculobullous disease caused by IgA autoantibodies in the basement membrane zone. An 84-year-old man was started on spironolactone two weeks before presentation for the management of hypertension and heart failure with preserved ejection fraction. He presented to our hospital for evaluation of worsening lower extremity swelling and a painful pruritic rash that started on the day preceding his presentation. On examination, he had 3+ lower extremity edema and an erythematous, painful, pruritic, bullous rash on all his extremities...

Linear IgA bullous dermatosis (LABD) is an acquired autoimmune subepidermal blistering skin disease characterized by circulating and tissue-bound IgA autoantibodies that recognize epitopes within the hemidesmosomal protein BP180, including its NC16A domain. Histologically, LABD has long been defined by neutrophil infiltration and dermal-epidermal separation. However, the pathogenic roles of anti-NC16A IgA and neutrophils in LABD, as well as their interactions, have not been thoroughly studied. We show that passive transfer of patient-derived anti-NC16A IgA induce clinical and histologic LABD pathology in humanized NC16A mice that are reconstituted locally or systemically with human neutrophils...

Linear immunoglobulin A bullous dermatosis (LABD) is an idiopathic or drug-induced vesiculobullous disease typically managed with dapsone or colchicine. We report a case of LABD successfully treated with rituximab in a patient who was intolerant to first-line therapies and recalcitrant to typical immunosuppressants. The patient was initially started on prednisone and mycophenolate mofetil which resulted in minimal response and disease progression. Improvement was seen after two infusions of rituximab 1000 mg at 2 weeks apart with planned maintenance therapy...

Linear IgA/IgG bullous dermatosis (LAGBD) is a relatively rare autoimmune bullous disease characterized by both IgA and IgG antibodies to basement membrane zone. The heterogeneity and pathogenesis of antibodies and the relationship between IgA and IgG in LAGBD have not been fully elucidated. We observed clinical, histological and immunological features of three LAGBD cases at different time points in the disease course. In our cohort, two cases showed IgA antibodies to epidermal antigens vanished when their lesions cleared after 3 months of treatment...

UNLABELLED: Linear IgA bullous dermatosis (LABD) is a rare acquired skin blistering autoimmune disease. It can be diagnosed by confirming the presence of a linear band of IgA at the dermoepidermal junction on direct immunofluorescence microscopy. LABD can be characterized by vesicular lesions, diffuse blisters, or even as a mimicker of Steven-Johnson syndrome. LABD may be caused by tumours, infections, or drugs (amiodarone, furosemide, phenytoin, however, vancomycin is the potential inciting drug in most reports)...

Linear IgA/IgG bullous dermatosis (LAGBD) is a rare autoimmune subepidermal bullous disorder characterized by linear deposition of concurrent IgA and IgG autoantibodies along the basement membrane zone (BMZ). The clinical features of LAGBD can be diverse, including tense blisters, erosions, erythema, crusting and mucosa involvement, while papules or nodules are generally absent. In this study, we present a unique case of LAGBD, which showed prurigo nodularis-like clinical appearance on physical examination, linear deposition of IgG and C3 along the basement membrane zone (BMZ) in direct immunofluorescence (DIF), IgA autoantibodies against the 97-kDa and 120-kDa of BP180 and IgG autoantibodies against the 97-kDa of BP180 by immunoblotting (IB), while BP180 NC16a domain, BP230, and laminin 332 were negative by enzyme-linked immunosorbent assay (ELISA)...

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...

Linear IgA disease (LAD) is an uncommon autoimmune blistering disease that has been associated with medications, malignancy, and other autoimmune diseases, such as ulcerative colitis (UC). In this case report, a patient with a history of UC developed characteristic LAD lesions. While dapsone is considered first-line therapy for LAD, the treatment team opted for an underutilized, plausibly less toxic, and more simplified treatment regimen with sulfasalazine, successfully utilizing the two distinct actions of sulfasalazine's components - sulfapyridine and 5-aminosalicylate (5-ASA) - to concurrently treat both the LAD and UC symptoms...

In the era of targeted treatments based on next generation sequencing (NGS) analysis, clinicians must be diligent in aligning patients with treatments giving them the best chance of survival while weighing the risk of toxicity caused by agents targeting specific gene mutations. In this case, we describe a patient with Epidermal Growth Factor Receptor (EGFR) exon 20 insertion mutation positive recurrent lung adenocarcinoma who received amivantamab and experienced severe dermatologic toxicity.

Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is an autoimmune condition with various triggers. Because of the lack of randomized controlled trials on LABD treatment, management options are mostly anecdotal. This paper provides a comprehensive review of treatment options from a literature review of reported treatments to arm clinicians with a guideline for the management of LABD in both pediatric and adult patients as well as those recalcitrant to first-line therapy (dapsone and steroids). We additionally illustrate an algorithm to use for the management of LABD to aid clinicians when faced with unique patient circumstances...

This is the case report of a previously healthy four-year-old girl with a history of upper airway infection that was treated with a β-lactam antibiotic. She was seen in the emergency department one month later with vesiculobullous lesions with clear content that were isolated or grouped in rosettes. Direct immunofluorescence showed baseline linear positivity for immunoglobulin A (IgA) (+) and fibrinogen-positive bullous content with absent remaining immunosera expression. The observed results were compatible with linear IgA bullous dermatosis...

Linear IgA bullous dermatosis (LABD) is a rare autoimmune bullous disease characterized by linear IgA deposition along the skin basal membrane. In children, LABD classically presents with a "cluster of jewels" appearance, whereas in adults the classic presentation is itchy papules with tense vesicles and bullae on an erythematous base. We report the case of a 41-year-old woman with LABD that we suspect was induced by acute myeloid leukemia presenting with multiple vesicles and bullae that coalesced, forming the typical clinical manifestation of LABD and confirmed with histopathological and direct immunofluorescence...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

Linear IgA bullous dermatosis (LABD) is  a rare autoimmune/inflammatory skin condition. Here, we report on a patient who developed treatment resistant LABD. At diagnosis, elevations of IL-6 and C-reactive protein in the blood and extreme elevations of IL-6 in LABD bullous fluid were seen. The patient responded well to tocilizumab (anti-IL-6 receptor) treatment.

BACKGROUND: Mucous membrane pemphigoid (MMP) with anti-laminin 332 autoantibodies may be associated with malignancies, however, current serological assays have considerable limitations. At present, no commercial test for anti-laminin 332 antibodies is available, restricting the diagnosis to specialized laboratories worldwide. Biochip immunofluorescence microscopy has shown promising results in selected cohorts of laminin 332-MMP patients. OBJECTIVES: To detect anti-laminin 332 antibodies by biochip immunofluorescence microscopy in a real-life cohort of MMP patients and compare the results with those from traditional immunoblotting...