Linear IgA bullous dermatosis of childhood: Retrospective single-center cohort.

Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disorder impacting children and adults. In this single-center retrospective chart review of pediatric patients with LABD at a large tertiary referral center, we report the unifying and unique clinical features of 10 pediatric patients. Patients typically presented with the "cluster of jewels" sign (n = 6; 60%), mucous membrane involvement (n = 5; 50%) and had a mean disease duration of 38 months; six patients (60%) required inpatient admission for management of their skin disease, including all five patients who had mucous membrane involvement. Our findings suggest that pediatric LABD may be a disease with high morbidity and may be associated with severe complications when mucous membranes are involved.

Drug-Induced Linear IgA Bullous Dermatosis in an Oncologic Patient.

Blister formation in the skin can result from various conditions, such as autoimmune disorders, drug reactions, infections, etc. A comprehensive patient assessment may offer clues for diagnosis. Linear IgA bullous dermatosis (LABD) is a rare subepidermal blistering disorder characterized by the deposition of IgA at the basement membrane zone of the skin and mucous membranes. Here, we describe a case of a patient with a new onset of painless blisters located in the skin and oral mucosa after initiating antibiotic treatment.

Rituximab for linear immunoglobulin A bullous dermatosis.

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.

IgA autoantibody may be the foremost pathogenic in three cases of linear IgA/IgG bullous dermatosis.

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. One refractory case showed increasing antigens targeted by IgA antibodies with the progression of the disease. Collectively, the results suggest that IgA antibodies may play a major role in LAGBD. In addition, epitope spreading may be related to disease relapse and treatment refractory.

Vancomycin-induced bullous dermatosis: a rare case report.

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). Case presentation: The authors present here a case of a 61-year-old woman with a history of HTN. The patient had a discectomy 15 years ago, and also underwent a lumbar fusion surgery that resulted in complications with her discitis. Due to the complications from the surgery, intravenous treatment with vancomycin and meropenem was initiated. After a few days of treatment, the patient developed clear, tense, fluid-filled bullae over the upper extremities. Immunofluorescence microscopy is not available in our hospital. Therefore a diagnosis of vancomycin-induced LABD was proposed based on the clinical manifestation of the lesions and the coincidence with vancomycin administration. After 2 days of discontinuing the administration of vancomycin and applying local diprosone, the lesions started to regress and a full recovery was achieved on day 10. Discussion and conclusion: Even though drug-induced LABD is uncommon, its incidence has been steadily increasing in the last few years. LABD is a simple condition with a good prognosis and full recovery after the discontinuation of vancomycin.

Azithromycin as a Possible Cause of Linear IgA Bullous Dermatosis.

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. Lesions resolved over two weeks with an oral prednisone taper and topical clobetasol. This case represents just one of two previously reported cases in the literature of azithromycin-associated LABD. While LABD is well known to be induced by certain medications, this is only the second report of it being associated with the use of a macrolide. We propose that macrolides be included as a potential cause of medication-induced LABD.

Successful Treatment of Linear IgA Disease and Ulcerative Colitis With Sulfasalazine.

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. The authors discuss the pathophysiology of LAD and UC and expound on the mechanistic theory of their association. Additionally, the pharmacodynamics of sulfasalazine and considerations of its side effect profile are examined.

Management Options for Linear Immunoglobulin A (IgA) Bullous Dermatosis: A Literature Review.

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.

Linear Immunoglobulin A Dermatosis: A Rare Case Illustrating Successful Treatment With Dapsone.

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 ß-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. After confirming the diagnosis and excluding glucose-6-phosphate dehydrogenase (G6PD) deficiency, dapsone was added to the initial treatment with systemic and topical corticosteroids. This case report is a reminder of the importance of a high index of clinical suspicion for this condition to reach a timely diagnosis.

A String of Pearls: Linear Immunoglobulin A (IgA) Bullous Dermatosis in the Setting of Imipramine Use and Newly Diagnosed Ulcerative Colitis.

Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is an autoimmune disease affecting children or adults that leads to subepithelial vesiculobullous lesions on the skin and/or mucosa. Due to the histologic and clinical appearance of the disease with tense and pruritic blisters, direct immunofluorescence is required for diagnosis, which features the characteristic linear deposition of IgA autoantibodies along the basement membrane zone. LABD can be idiopathic, drug-induced, or associated with a systemic disease such as inflammatory bowel disease. Many drugs have been implicated, such as antibiotics, anti-hypertensives, anti-epileptics, analgesics, and immunosuppressive medications. Treatment of LABD centers on discontinuation of the offending drug, if applicable, as well as pharmacotherapy with dapsone as the first-line treatment. Adjunctive therapy with sulphonamides, systemic corticosteroids, cyclosporine, colchicine, intravenous immunoglobulins, tetracyclines, erythromycin, and dicloxacillin has also shown benefits. We report the case of a young adult patient who developed LABD with a background of recent initiation of treatment with imipramine and newly diagnosed ulcerative colitis.

Eosinophil-rich linear IgA bullous dermatosis induced by mRNA COVID-19 booster vaccine.

We present a case of eosinophil-rich linear IgA bullous disease (LABD) following the administration of a messenger RNA COVID-19 booster vaccine. A 66-year-old man presented to the emergency department with a 3-week history of a pruritic blistering rash characterized by fluid-filled bullae and multiple annular and polycyclic plaques. He was initially diagnosed with bullous pemphigoid based on a biopsy showing a subepidermal blister with numerous eosinophils. However, direct immunofluorescence studies showed linear IgA and IgM deposition along the basement membrane zone with no immunoreactivity for C3 or IgG. Additionally, indirect immunofluorescence was positive for IgA basement membrane zone antibody. The patient was subsequently diagnosed with LABD and initiated on dapsone therapy with resolution of his lesions at 3-month follow-up. This case illustrates the growing number of autoimmune blistering adverse cutaneous reactions from vaccination. Dermatopathologists should be aware that features of autoimmune blistering diseases can overlap and may not be distinguishable based on these histopathological findings alone. Confirmation with direct immunofluorescence and/or serological studies may be necessary for accurate diagnosis.

Linear IgA bullous dermatosis in the setting of angioimmunoblastic T-cell lymphoma.

We report an 80-year-old male developing linear IgA bullous dermatosis (LAD) in the setting of angioimmunoblastic T-cell lymphoma (AITL). This phenomenon is rare, as only three cases have been described in the literature. The pathophysiologic process can be attributed to dysregulation in somatic hypermutation and the expression of chemokine receptor 5 in AITL, contributing to increased IgA. Immunoglobulin production resulting from clonal plasma cell expansion may be because of the B-cell promotional effect by neoplastic follicular helper T-cells. Beyond providing a pathophysiologic platform for AITL-associated LAD, we also briefly summarized prior cases. This report demonstrates the importance of considering LAD in the differential diagnosis for patients with a bullous eruption in the setting of AITL.