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.

Case Report: Prurigo nodularis-like linear IgA/IgG bullous dermatosis: a case report and literature review.

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). After administration of minocycline, the skin lesions improved. We performed a literature review of LAGBD cases with heterogeneous autoantibodies and found clinical presentations of most cases resemble bullous pemphigoid (BP) and linear IgA bullous disease (LABD), which is consistent with previous reported findings. We aim to increase our understanding of this disorder and to enhance the importance of applying immunoblot analyses and other serological detection tools in clinic for precise diagnosis as well as accurate treatment strategy of various autoimmune bullous dermatoses.

A propósito de un caso: enfermedad IgA lineal de la infancia / Apropos of a case: Linear IgA bullous dermatosis of childhood

La enfermedad IgA lineal se trata de un trastorno autoinmune poco frecuente. En la infancia aparece sobre todo en edad preescolar, y en adultos es necesario realizar un buen diagnóstico diferencial con otras enfermedades ampollosas como la dermatitis herpetiforme o el penfigoide ampollar. Hasta un cuarto de los casos se relacionan con infecciones o con la toma de medicamentos entre los que destacan antibióticos y antiinflamatorios no esteroideos. El diagnóstico se basa en la clínica, biopsia cutánea e inmunofluorescencia. Típicamente se presenta como ampollas anulares tensas de morfología anular con imagen característica en collar de perlas. Al realizar el estudio de inmunofluorescencia directa se evidencia una banda lineal de IgA en la unión dermoepidérmica. Suele responder de forma completa a la retirada del fármaco causante y al tratamiento con Dapsona oral y glucocorticoides tópicos. (AU)

Linear IgA bullous dermatosis in an acute myeloid leukemia patient: a rare case report.

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. The patient was treated with a combination of oral and topical corticosteroids with excellent results.

A Case of Successful Management of Adult-Onset Linear IgA Bullous Disease With Sulfasalazine During the COVID-19 Pandemic.

Linear IgA bullous disease (LABD) is a rare, acquired, autoimmune, pruritic, and blistering skin condition. Dapsone is a first line treatment option, however, there are limited options if this fails, or if contraindicated. We present a case of successful management of LABD with sulfasalazine. A 46-year-old Caucasian female with LABD was commenced on high dose corticosteroids. She failed to wean, and dapsone was contraindicated due to a history of primary sclerosing cholangitis and risk of hepatitis. Following the failure of mycophenolate mofetil, sulfasalazine was trialed and successfully controlled both this patient’s LABD and ulcerative colitis. There is little literature on the use of sulfasalazine in dermatological conditions. We present sulfasalazine as an option for patients who are unable to use classically used treatments for LABD, or in those who have a dual diagnosis, as in this case, allowing for one agent to manage both conditions. Furthermore, The National Institute for Health and Care Excellence guidance mentions sulfasalazine as one of the few drugs that can be continued during the COVID-19 pandemic, and its use spared this patient from the significant immunosuppression associated with other treatment modalities.J Drugs Dermatol. 2022;21(12): doi:10.36849/JDD.6717.

Disseminated Tuberculosis in a Nigerian Adolescent with Linear IgA Bullous Dermatosis: A Case Report and Review of Literature.

Linear IgA bullous dermatosis (LABD) is an auto-immune disease affecting young children and adults, characterized by the linear deposition of IgA at the basement membrane zone with resultant complement activation and a cascade of immune reactions. There is a loss of adhesion at the dermo-epidermal junction and subsequent blister formation. It is a rare disease that has a good prognosis with adequate therapy. However, the underlying depressed immunity associated with the disease may expose them to such infections as tuberculosis. We report the case of an 11-years-old Nigerian female adolescent with LABD, diagnosed at the age of four years but defaulted on follow-up, who developed disseminated tuberculosis (pulmonary, lymph nodes, abdominal and pericardial effusion) seven years after the appearance of the initial blistering skin lesions. She commenced anti-tuberculosis drugs, steroids, and a tube pericardiostomy for the pericardial effusion. Dapsone was initiated for the LABD during the continuation phase of anti-tuberculosis therapy, with subsequent disappearance of the skin rash within two weeks.

La dermatose bulleuse linéaire à IgA (DBL) est une maladie auto-immune affectant les jeunes enfants et les adultes, caractérisée par le dépôt linéaire d'IgA dans la zone de la membrane basale, avec l'activation du complément qui en résulte et une cascade de réactions immunitaires. Il y a une perte d'adhérence à la jonction dermo-épidermique et une formation ultérieure de vésicules. C'est une maladie rare qui a un bon pronostic avec un traitement adéquat. Cependant, l'immunité déprimée sous-jacente associée à la maladie peut les exposer à des infections telles que la tuberculose. Nous rapportons le cas d'une adolescente nigériane de 11 ans atteinte de la LABD, diagnostiquée à l'âge de quatre ans mais en défaut de suivi, qui a développé une tuberculose disséminée (pulmonaire, ganglions lymphatiques, épanchement abdominal et péricardique) sept ans après l'apparition des lésions cutanées vésiculeuses initiales. Elle a commencé à recevoir des médicaments antituberculeux, des stéroïdes et une péricardiostomie par sonde pour l'épanchement péricardique. La dapsone a été initiée pour la DLB pendant la phase de continuation du traitement antituberculeux, avec une disparition de l'éruption cutanée en deux semaines. Mots clés: IgA linéaire, dermatose bulleuse, tuberculose disséminée, adolescent.

Ulcerative colitis complicated with linear immunoglobulin A bullous dermatosis.

Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is a rare disorder involving subepidermal blistering characterised by IgA deposition along the basement membrane. The clinical features of LABD are variable but can include bullae, vesicles and erythematous lesions. Histopathology reveals formation of subepidermal bullae and linearly deposition of IgA in the basement membrane of the epidermis. LABD has been reported as a rare complication of ulcerative colitis (UC). We report the case of a young woman with UC complicated by LABD. The latter manifested as vesicles with erythema on almost the entire body. A biopsy of the skin lesions revealed linear IgA deposits in the basement membrane according to a direct immunofluorescence assay. Prednisolone administration resulted in clinical remission of UC but poor improvement of skin lesions. Oral administration of diaminodiphenyl sulfone led to improvement of blisters. Thereafter, abdominal and skin symptoms did not recur and she was discharged from hospital.

Pyoderma gangrenosum occurring 7 years after onset of linear IgA bullous dermatosis in a young woman.

We present a case of coexistence of pyoderma gangrenosum (PG) and linear IgA bullous dermatosis (LABD), with a 7-year interval between them. This is the first case of coexisting PG and LABD, to our knowledge.

Linear IGA bullous dermatosis potentially triggered by vaccination.

Linear IgA bullous dermatosis (LABD) is a mucocutaneous autoimmune blistering disease affecting both adults and children. It is caused by IgA antibodies targeting multiple antigens along the basement membrane zone, leading to disruption of dermoepidermal junction and development of bullous lesions which often presents in characteristic arrangement. Although most LABD cases have been reported to be idiopathic, different triggers have been described, including several drugs and infection. However, the occurrence of vaccine-induced cases of LABD is not widely known and accepted due to the few reports available. We present two cases of LABD occurred following different triggers, rising the suspicion for a possible pathogenetic role of vaccines.

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

IMPORTANCE: 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. OBJECTIVE: 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. DESIGN, SETTING, AND PARTICIPANTS: 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. MAIN OUTCOMES AND MEASURES: The clinical course of IgA EBA was observed before and during different treatments. RESULTS: 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. CONCLUSIONS AND RELEVANCE: 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.