Linear Immunoglobulin A Bullous Dermatosis Research Articles

Linear IgA Bullous Dermatosis in Korea Using the Nationwide Health Insurance Database.

(1) Background: Linear immunoglobulin A bullous dermatosis (LABD) is a rare autoimmune, subepidermal blistering disease, characterized by linear IgA deposits along the epidermal basement membrane. LABD is idiopathic and is associated with medication and systemic autoimmune diseases. (2) Methods: We investigated the demographic characteristics, disease course, causative agents, and associated diseases in Korean patients with LABD. The Korean Health Insurance Review and Assessment Service database was used to obtain data. We identified 670 LABD cases between 2010 and 2022. (3) Results: The annual incidence of LABD was 1.3 per 100,000 persons, with a higher prevalence in individuals ≥60 years old. The patients were treated with dapsone for 30.7 ± 56.7 days, had 1.3 ± 0.7 hospital visits, and were hospitalized for 19.8 ± 19.7 days. Risk factors, including malignancy, commonly preceded LABD. Antibiotic use, specifically vancomycin and third-generation cephalosporins, was a risk factor. The mean age of LABD diagnosis was 55.9 ± 21.7 years. (4) Conclusion: This is the first published study to assess a nationwide cohort for LABD. The incidence of LABD was higher than that in other studies. Most case reports have linked LABD with the administration of specific antibiotics; however, this study shows there were more associations with other conditions.

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.

Evaluation and Comparison of Clinical and iLaboratory Characteristics of Patients With IgA Epidermolysis Bullosa Acquisita, Linear IgA Bullous Dermatosis, and IgG Epidermolysis Bullosa Acquisita

Immunoglobulin A (IgA) epidermolysis bullosa acquisita (EBA) is an autoimmune skin blistering disease with IgA autoantibodies directed against type VII collagen. There is debate whether it should be considered part of the clinical spectrum of linear IgA bullous dermatosis (LABD) or a separate disease entity. This cohort study aimed to define the clinical features and treatment responses of IgA EBA and anti-BP180-driven LABD, and to compare the prevalences of IgA EBA anti-BP180 LABD and classic IgG-mediated EBA in an autoimmune diagnostic laboratory database. This retrospective cohort study and case series study included demographic, immunopathologic, and serologic data from 300 patients diagnosed with IgA EBA, IgG EBA, or LABD. Furthermore, clinical features and treatment responses of IgA EBA were analyzed in a case series including 4 patients with IgA EBA. All patients from the database of the autoimmune diagnostic laboratory at the Department of Dermatology, University of Lübeck, Germany, who were diagnosed with IgA EBA, LABD, or IgG EBA between October 2010 and July 2019 were included. Four patients diagnosed with IgA EBA between October 2015 and January 2018 are described in detail. The clinical course of IgA EBA was observed before and during different treatments. The database search yielded 21 cases of IgA EBA (12 females [57%]/9 males [43%]), 222 cases of LABD (111 females [51%]/106 males [49]), and 57 cases of IgG EBA (29 females [50%]/28 males [48%]). The median (range) age of each cohort was 64 (4-81) years for IgA EBA, 56 (3-92) years for IgG EBA, and significantly older compared with IgG EBA (P = .002) for those with LAPD (median [range], 70 [1-94] years). The patients with IgA EBA exhibited heterogeneous clinical presentations that significantly differed from those of anti-BP180 LABD. All 4 patients with IgA EBA described in detail were first treated with dapsone, but only 1 responded to this treatment. The others required treatment with high-dose dexamethasone, rituximab, and/or intravenous immunoglobulins to achieve partial clinical remission. Overall, the findings of this cohort study and small case series suggest that IgA EBA may be more common than expected and may require more intensive systemic treatment than LABD, suggesting it should be considered a separate disease entity.

Mixed immunobullous disease in infants: Falls in bullous pemphigoid-linear IgA spectrum?

Bullous pemphigoid (BP) and linear immunoglobulin A bullous dermatosis (LABD) are common immunobullous diseases affecting both adults and children, with minor differences in clinical presentation. The only test for a definitive diagnosis is direct immunofluorescence (DIF) study, with linear deposits of immunoglobulin G (IgG) (mainly) at the dermoepidermal junction (DEJ) being diagnosed as BP and linear deposits of IgA at DEJ, diagnosed as LABD. Mixed immunobullous disease is the term given for immunobullous diseases with equal IgA/IgG deposits at the DEJ. Through this case report, a mixed immunobullous disease with clinical diagnosis of LABD and DIF findings of BP is being discussed. Furthermore, the presence of a BP-LABD spectrum of disease that can progress in either direction and present with features of both diseases is discussed.

An Unusual Case of Drug-Induced Linear IgA Bullous Disease

An Unusual Case of Drug-Induced Linear IgA Bullous Disease

Linear IgA Bullous Dermatosis Associated with Ulcerative Colitis in a 14-Year-Old Boy: A Case Report

Introduction: Linear immunoglobulin A bullous dermatosis (LABD) is a rare, heterogeneous, autoimmune blistering skin disorder that can be associated with other autoimmune conditions. Case presentation: We describe the case of a 14-year-old Italian boy with previously diagnosed ulcerative colitis who presented a severe LABD well controlled after appropriate medical therapy and in total remission after a follow-up of one year.

Linear immunoglobulin-A bullous dermatosis complicating multi-organ tuberculosis at early-stage treatment

Linear immunoglobulin-A bullous dermatosis complicating multi-organ tuberculosis at early-stage treatment

Drug-induced linear IgA bullous dermatosis after discontinuation of cefuroxime axetil treatment

Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is a rare autoimmune blistering disorder. The disease may be either idiopathic or druginduced. Over the past 30 years, approximately one hundred LABD cases have been described as induced by a wide range of drugs, chiefly antibiotics. We report the case of 37-year-old woman who developed pruritic bullous lesions spread all over the body three weeks after her last dose of cefuroxime axetil. Antibiotic therapy was started due to rhino-sinusitis. In most reported cases of drug-induced LABD, skin lesions occur within the time of drug administration. However, the onset of disease may be even after discontinuation of treatment. It seems that in such cases, other clinical conditions (like infection) act, as cofactors of immunologic response.

Neonatal Linear Immunoglobulin A Bullous Dermatosis: A Rare Presentation

Linear immunoglobulin A bullous dermatosis (LABD) is an autoimmune blistering disease that most commonly presents in preschool-aged children. There have been few neonatal reports, all of which had life-threatening aerodigestive complications requiring mechanical intervention and systemic therapy. We present a case of LABD in a neonate who had an uncomplicated course and was treated conservatively with only low-potency topical corticosteroids and wound care before resolution of his skin lesions.

Linear IgA bullous dermatosis: a clinical study of 16 cases at National Taiwan University Hospital

Linear immunoglobulin A bullous dermatosis (LABD) is a rare autoimmune subepidermal bullous disease. It is defined by continuous linear deposition of IgA in the basement membrane zone on direct immunofluorescence microscopy. The clinical presentations of LABD may mimic other diseases, and data in Taiwanese populations are still lacking. The current study aims to examine LABD status in Taiwan. We reviewed the database at our institute from 1995 to 2008. The gold standard for the diagnosis of LABD is based on continuous linear depositions of IgA in the basement membrane zone on direct immunofluorescence. A total of 16 LABD patients were identified. Mean age at diagnosis was 55 years, and most (> 80%) occurred after the fourth decade. The trunk was most commonly involved (76%). However, in contrast to previous reports, the mucosal involvement was rare in our series (18%). Initial impressions were dermatitis herpetiformis in 8 patients (50%), bullous pemphigoid in 4 patients (25%), and vasculitis, varicella, and pemphigus vulgaris in the remaining 4 patients (25%). Four patients reported a history of drug ingestion shortly before the onset of the disease, and all recovered after discontinuing the offending drugs. One of them had griseofulvin-associated LABD, a case not reported previously. The other three drugs were rifampin, vancomycin and gemcitabine. Among the various regimens, dapsone (100 mg) twice a day achieved the best treatment response in the five treated patients. The rare and diverse presentations of LABD highlight the importance of our study results in aiding clinical diagnosis and planning treatment strategies.

Linear immunoglobulin A bullous dermatosis

Linear immunoglobulin A bullous dermatosis (LABD) is a rare acquired, heterogeneous, subepidermal blistering disease characterized by linear IgA deposits at the dermal–epidermal basement membrane zone, often with circulating IgA antibodies in the basement membrane zone. Linear IgA disease shares many characteristics with other subepidermal bullous diseases, mainly bullous pemphigoid and dermatitis herpetiformis. However, there are clinical and immunopathologic distinctions that have led to the recognition of LABD as a separate entity. This review will focus on the clinical manifestations, the etiopathogenesis, as well as the management of LABD, based on the best data available at the time of preparation.

A case of linear immunoglobulin A bullous dermatosis in a patient exposed to sun and an analgesic

Background: Medications are the most common triggers of linear immunoglobulin A bullous dermatosis (LABD). LABD induced by ultraviolet (UV) radiation has rarely been described. This article reports a case of LABD in a patient exposed simultaneously to an analgesic and UV radiation. Case summary: A 45-year-old woman developed LABD lesions on sun-exposed skin after 3 days of sunbathing and consumption of a medication for headache containing propyphenazone, butalbital, and caffeine. The lesions spread to unexposed skin and, by day 5, the patient had vesicles and bullae on the palms and soles, face, trunk, and extremities. LABD was diagnosed with direct and indirect immunofluorescence microscopy and Western blot analysis. Treatment was successful with prednisone, started at a dosage of 1 mg/kg/d, for 5 months. Lesions located on sunexposed areas, the absence of relapse for 5 years despite continuing sun exposure against medical advice, and subsequent avoidance of the suspected medication suggest that the bullous flare may have been due to the concomitant action of 2 triggers. That the analgesic had a role in this cutaneous manifestation is possible according to the Naranjo algorithm for adverse drug reactions. Conclusions: A case of LABD possibly associated with sun exposure and an analgesic is described. Treatment with prednisone successfully resolved the lesions in this patient.

Amoxicillin–clavulanic acid‐induced linear immunoglobulin A bullous dermatosis: case report and review of the literature

Linear immunoglobulin A bullous dermatosis (LABD) is an autoimmune subepidermal blistering disease, rarely induced by drugs. We describe a case of a 47-year-old man who developed a severe blistering eruption after therapy with amoxicillin-clavulanic acid. Histopathologic examination and direct immunofluorescence were consistent with a diagnosis of LABD. Therapy with dapsone controlled the disease but, after sun exposure, there was a worsening of the illness. To our knowledge, this is the first case of amoxicillin-clavulanic acid-induced LABD in an adult.

Drug-Induced Linear Immunoglobulin-A Bullous Dermatosis

Studies on the target antigens in idiopathic LABD have identified a number of different antigens, including a 97-kDa antigen shown to be an anchoring filament; two antigens corresponding to the bullous pemphigoid (BP)-associated antigen and the epidermolysis bullosa acquisita (EBA)-associated antigen;20–23 and a number of other antigens including a 285-kDa24,25 and a 255kDa26 antigen (Fig 1). These findings suggest the heterogeneity of idiopathic LABD. Recently, the target antigens of three patients with drug-induced LABD have been identified.18 Immunoelectron microscopy showed the antibodies to be localized in the lamina lucida, in both the lamina lucida and the lamina densa, and in both the lamina densa and sublamina densa regions. Western immunoblotting of these patients’ serum identified an IgA that reacted to a 230-kDa protein that co-migrated with the BP-associated antigen in one patient and an IgA that reacted with a 97-kDa antigen in a second patient, this being presumably the anchoring filament antigen detected in the studies on idiopathic LABD.

Linear IgA bullous dermatosis of adults

The skin disorder designated as linear IgA bullous dermatosis (LABD), identified in 1979 (l), had been for many years a matter of controversy. This controversy was due mainly to its clinical diversity, as some cases closely resemble dermatitis herpetiformis (DH), some cases are indistinguishable from bullous pemphigoid (BP), and still other cases combine features of both (2). No wonder that some authors considered this disease as DH or its variety (3 6), others as BP (7,8), and yet others as intermediate or mixed forms of DH and BP (2,9). In support of the relationship with DH were the skin immune deposits of immunoglobulin A (IgA) class and, as a rule, a dramatic response to sulfones. Supporting the relationship with BP were the linear pattern of IgA deposits along the basement membrane zone (BMZ) and, occasionally, the concomitant presence of complement in the same distribution (1,2,9). Furthermore, at least in some cases, the ultrastructural depositions of IgA, in the lamina lucida, similar to that in BP, suggested some relationship between LABD and BP (10 12). In a proportion of cases of LABD, the linear IgA deposits are found below the basal lamina (12-14) or in both locations (6,11,12,15). The main difference from BP is the IgA-class skin deposits, papillary microabscesses in the histology, and, as a rule, a good response to sulfones. The most important reason for separation of LABD from DH is that LABD is not associated with gluten-sensitive enteropathy and, as could be expected, a glutenfree diet is not effective treatment for LABD (16). Recognition of LABD as a distinct entity, despite the clear-cut differences from DH and BP, took a long time until more data on immunoelectron microscopy, serology, genetics,