Neonatal linear IgA bullous dermatosis / 中华皮肤科杂志

A 10-day-old male infant presented with skin erythema and blisters for 6 days. Skin examination showed scattered or confluent erythema all over the body, tense blisters of varying sizes on the normal skin or an erythematous base, and some blisters were ulcerated and erosive; bloody bullae and erythematous erosive patches could be seen on the oral mucosa. Histopathological examination revealed subepidermal blisters, and there were some neutrophils and a few eosinophils in the blisters. Direct immunofluorescence assay showed homogeneous linear IgA and granular C3 deposits along the basement membrane zone, without IgG deposits. The diagnosis of neonatal linear IgA bullous dermatosis was confirmed. After comprehensive treatments including nutritional support and anti-infection treatment, skin erythema and blisters subsided, and the mucosal damage was attenuated. The telephone follow-up 16 months after discharge showed that the infant was in good general condition with normal growth and development, and the oral mucosal lesions had subsided and healed, without new skin lesions.

Dermatosis ampollar por IgA lineal inducida por Vancomicina: reporte de un caso / Vancomycin-induced linear igabollous dermatosis: a case report

RESUMEN La dermatosis ampollar por IgA lineal del adulto (DLA) es una enfermedad autoinmune adquirida infrecuente, caracterizada por el depósito lineal de anticuerpos IgA en la membrana basal. La mayoría de los casos reportados son de causa idiopática, pero esta entidad también se ha visto asociada a ciertos fármacos, siendo la vancomicina el más frecuente. Se presenta un caso de DLA asociada a vancomicina, con extensa afectación cutánea y compromiso mucoso, tratado con dapsona y corticoides sistémicos con buena respuesta.

ABSTRACT Adult linear IgA bollous dermatosis (LABD) is a rare acquired autoimmune disease characterized by linear deposition of IgA antibodies on the basement membrane. Most of the reported cases are of idiopathic cause, but this entity has also been associated with certain drugs, vancomycin being the most frequent. We present a case of LABD associated with vancomycine, with extensive skin and mucosal involvement, treated with dapsone and systemic corticosteroids with a good response.

Linear IgA bullous dermatosis / 中华皮肤科杂志

Linear IgA bullous dermatosis (LABD) is a rare autoimmune subepidermal blistering disease. Currently, researches on LABD are still limited, and most are case reports. This review summarizes research advance in etiology and pathogenesis, clinical and histopathological manifestations, diagnosis and treatment of LABD.

Consensus on the treatment of autoimmune bullous dermatoses: dermatitis herpetiformis and linear IgA bullous dermatosis - Brazilian Society of Dermatology

Abstract: Dermatitis herpetiformis and linear IgA bullous dermatosis are autoimmune diseases that present with pruritic urticarial papules and plaques, with formation of vesicles and blisters of subepidermal location, mediated by IgA antibodies. Mucosal lesions are present only in linear IgA bullous dermatosis. The elaboration of this consensus consisted of a brief presentation of the different aspects of these dermatoses and, above all, of an updated literature review on the various therapeutic options that were discussed and compared with the authors' experience, aiming at the treatment orientation of these diseases in Brazil. Dermatitis herpetiformis is a cutaneous manifestation of celiac disease, and can be controlled with a gluten-free diet and dapsone. On the other hand, linear IgA bullous dermatosis arises spontaneously or is triggered by drugs, and can be controlled with dapsone, but often requires the association of systemic corticosteroids and eventually immunosuppressants.

BP180-related autoimmune blistering diseases / 中华皮肤科杂志

BP180-related autoimmune blistering diseases include bullous pemphigoid,lichen planus pemphigoides,linear IgA bullous dermatosis,pemphigoid gestationis and cicatricial pemphigoid.There are multiple autoantibody-reactive sites on the extracellular region of BP180.Current studies show that there is heterogeneity in the autoimmune blistering disease-related target sites on BP 180,and different clinical manifestations of the same disease are related to the heterogeneity of target sites.However,further studies and analysis are still needed for the mechanism of the heterogeneity.

A Case of Vancomycin-induced Linear IgA Bullous Dermatosis / 대한피부과학회지

No abstract available.

Adult linear IgA bullous dermatosis: report of three cases

Abstract: Linear immunoglobulin A bullous dermatosis is a rare autoimmune disease that usually has an excellent prognosis in childhood; however, its control is more difficult in adults. It presents heterogeneous clinical manifestations and is frequently confused with other bullous diseases such as bullous pemphigoid and Duhring's dermatitis herpetiformis. Dermatologists' awareness of this disease contributes to early diagnosis and appropriate treatment. We thus report three cases of linear immunoglobulin A dermatosis in adults.

Piperacillin-Tazobactam-Induced Linear IgA Bullous Dermatosis Supported by a T-Cell Activation Assay

Linear immunoglobulin (Ig) A bullous dermatosis (LABD) is a rare subepidermal autoimmune blistering disease characterized by linear IgA deposits at the basement membrane zone visualized with direct immunofluorescence (DIF). Most cases of LABD are idiopathic, but some are drug-induced with vancomycin being the most common causative agent. We herein report a patient presenting with blisters and erosive lesions, primarily in the intertriginous and flexor areas, consistent with a diagnosis of piperacillin-tazobactam-induced LABD based on the patient's clinical course and histopathology, DIF, and in vitro T-cell activation assay (TAA) findings. Only one case of piperacillin-tazobactam-induced LABD has been previously reported. In addition to its rarity, our case was also unique in that the skin lesions occurred in the intertriginous and flexor areas, uncommon locations for typical adult patients with LABD, and TAA strongly suggested an association with the causative drug.

Vancomycin-associated linear IgA disease mimicking toxic epidermal necrolysis

Abstract Linear IgA dermatosis is a rare subepidermal autoimmune blistering disease characterized by linear deposition of IgA along the basement membrane zone. In the last three decades, many different drugs have been associated with the drug-induced form of the disease, especially vancomycin. We report a case of vancomycin-induced linear IgA disease mimicking toxic epidermal necrolysis. The aim of this work is to emphasize the need to include this differential diagnosis in cases of epidermal detachment and to review the literature on the subject and this specific clinical presentation.

Linear IgA and IgG bullous dermatosis

Abstract Childhood linear immunoglobulin A dermatosis is a rare autoimmune vesiculobullous disease. It results in linear deposition of autoantibodies (immunoglobulin A) against antigens in the basal membrane zone, leading to subepidermal cleavage. Additional depositions of immunoglobulin G and complement-3 might occur. It is still debated whether concomitant findings of immunoglobulins A and G should be considered a subtype of this dermatosis or a new entity. Further studies are needed to recognize this clinical variant.

Concurrent Drug-Induced Linear Immunoglobulin A Dermatosis and Immunoglobulin A Nephropathy

Diseases associated with immunoglobulin A (IgA) antibody include linear IgA dermatosis, IgA nephropathy, Celiac disease, Henoch-Schonlein purpura, etc. Although usually idiopathic, IgA antibody is occasionally induced by drugs (e.g., vancomycin, carbamazepine, ceftriaxone, and cyclosporine), malignancies, infections, and other causes. So far, only a few cases of IgA bullous dermatosis coexisting with IgA nephropathy have been reported. A 64-year-old female receiving intravenous ceftriaxone and metronidazole for liver abscess had purpuric macules and papules on her extremities. One week later, she had generalized edema and skin rash with bullae and was diagnosed with concurrent linear IgA dermatosis and IgA nephropathy. After steroid treatment, the skin lesion subsided within two weeks, and kidney function slowly returned to normal. As both diseases occurred after a common possible cause, we predict their pathogeneses are associated.

Clinical Study of Korean Patients with Linear IgA Bullous Dermatosis / 대한피부과학회지

BACKGROUND: Linear IgA bullous dermatosis (LABD) is an autoimmune, chronic bullous disease characterized by sub-epithelial bullae with linear IgA deposits along the basement membrane. LABD primarily affects young children and adults. There has been no study on LABD in Korea to date. OBJECTIVE: The purpose of this study was to evaluate the clinical features, laboratory examinations, treatments, and outcomes of Korean LABD patients. Patient characteristics including age at disease onset, gender, medical associations, medications, immunofluorescence findings, disease duration, treatment, and outcome were analyzed. METHODS: A retrospective analysis was conducted on 16 LABD patients diagnosed at Gangnam Severance Hospital between 1999 and 2014. RESULTS: A total of 16 LABD patients were included in the study, 5 children and 11 adults. The mean ages at disease onset in children and adults were 3.2 and 41 years, respectively. Eighty percent of children with LABD showed complete remission. In adults, partial remission was achieved in 36.4%, and complete remission in 54.5% of patients. Two patients were diagnosed with drug-induced LABD, and 2 with ulcerative colitis-associated LABD. CONCLUSION: Our report differed from previous reports in that all 5 children with LABD were male, and the incidence in adults was higher in females than males. Most patients responded well to dapsone and oral prednisolone. Since LABD is rare and can be misdiagnosed as impetigo or bullous pemphigoid, diagnosis by immunofluorescence microscopy is necessary for proper treatment to attain disease remission.

Immunofluorescence testing in the diagnosis of autoimmune blistering diseases: overview of 10-year experience

BACKGROUND: Immunofluorescence testing is an important tool for diagnosing blistering diseases. OBJECTIVE: To characterize the immunofluorescence findings in patients diagnosed with autoimmune blistering skin diseases. METHODS: We retrospectively analyzed immunofluorescence results encompassing a 10-year period. RESULTS: 421 patients were included and divided into 2 groups: group 1- intraepidermal blistering diseases (n=277) and 2- subepidermal blistering diseases (n=144). For group 1, positive DIF findings demonstrated: predominance of IgG intercellular staining (ICS) and C3 for pemphigus foliaceus-PF (94% and 73% respectively), pemphigus vulgaris-PV (91.5%-79.5%) and paraneoplastic pemphigus-PNP (66%-33%); ICS IgA in 100% of IgA pemphigus cases, and IgG deposits in the basement membrane zone (BMZ) along with ICS in one Hailey-Hailey patient. The IIF findings revealed mean titers of 1:2.560 for PV and 1:1.280 for PF. For paraneoplastic pemphigus, IIF was positive in 2 out of 3 cases with rat bladder substrate. In group 2, positive DIF findings included multiple deposits at basement membrane zone for epidermolysis bullosa acquisita-EBA (C3-89%,IgG-79%,IgA-47%,IgM-21%) mucous membrane pemphigoid-MMP (C3,IgG,IgA,IgM-80%) and bullous pemphigoid-BP (C3-91%,IgG-39%,IgA-11%,IgM-6%), and IgA at basement membrane zone for IgA linear disease (99%) and dermatitis herpetiformis-DH (dermal papillae in 84.6%). For lichen planus pemphigoides, there was C3 (100%) and IgG (50%) deposition at basement membrane zone. indirect immunofluorescence positive findings revealed basement membrane zone IgG deposits in 46% of BP patients, 50% for EBA, 15% for IgA linear dermatosis and 50% for LPP. Indirect immunofluorescence positive results were higher for BP and EBA with Salt-Split skin substrate. CONCLUSION: Our results confirmed the importance of immunofluorescence assays in diagnosing autoimmune blistering diseases, and higher sensitivity for indirect ...

Dermatosis IgA lineal de la infancia: reporte de un caso / Linear IgA bullous dermatosis of childhood: case report

La dermatosis IgA lineal de la infancia es una infrecuente enfermedad vesiculoampollar adquirida de etiología autoinmune, caracterizada por los depósitos de IgA en una banda lineal en la membrana basal dermoepidérmica, que se observan con la técnica de inmunofluorescencia directa. Las lesiones características son vesículas y ampollas serosas tensas, por lo general, agrupadas dando un aspecto de "conjunto de joyas". El diagnóstico diferencial debe establecerse principalmente con otras dermatosis autoinmunes, como la dermatitis herpetiforme y el penfigoide buloso. El tratamiento de primera línea es la dapsona, con excelente respuesta a corto plazo. La enfermedad es benigna y tiende a cursar en brotes hasta su resolución espontánea. Presentamos el caso de una niña de 5 años que consulta por lesiones ampollares; se diagnosticó una dermatosis IgA lineal de la infancia, con excelente respuesta al tratamiento con dapsona en menos de 2 semanas.

Linear IgA bullous dermatosis is a rare acquired autoinmune vesiculobullous disease characterized by linear IgA deposit on the dermo-epidermal basement membrane observed with direct inmunofluorescence. The characteristic lesions are vesicles and tense serous bullae, which most often are grouped giving a "cluster of jewels" appearance. Differential diagnosis must be established with other autoimmune dermatosis, such as dermatitis herpetiformis and bullous pemphigoid. Dapsone is the first line therapy, with excellent response in a short period. This is a benign disease that tends to wax and wane in severity until it disappears spontaneously. We report the case of a 5-year-old girl presenting with bullous lesions, being diagnosed a linear IgA bullous dermatosis, with excellent response to dapsone in less than 2 weeks.

A Case of Chronic Bullous Disease of Childhood That Was Reactive to the Antigen of 120 kDa (LAD-1)

Chronic bullous disease of childhood (CBDC) is an autoimmune blistering disease that is characterized by Immunoglobulin A (IgA) deposits at the basement membrane zone. IgA autoantibodies (aAbs) from the serum of patients with CBDC react with antigens of 97 kDa (LABD97) and 120 kDa (LAD-1), and both of which are fragments of the extracellular domain of bullous pemphigoid 180 (BP180, type XVII collagen). The CBDC sera reacts with the immunodominant NC16a domain of BP180, which is the major region recognized by IgG aAbs in patients with bullous pemphigoid. A five-year-old boy presented with multiple pruritic tense blisters on the umbilical and inguinal areas for six weeks. The direct immunofluorescence of the perilesional area demonstrated linear deposits of IgA at the basement membrane zone. Using immunoblotting and an enzyme linked immunosorbent assay (ELISA), we identified the IgA aAbs reactive to antigens with a molecular weight of 120 kDa (LAD-1), which is a fragment of the extracellular domain of BP180.

Vancomycin-induced Linear IgA Bullous Dermatosis: A Case Report and Review of the Literature

Linear IgA bullous dermatosis (LABD) is a rare autoimmune bullous disease that can either occur without any apparent cause or be induced by the administration of certain drugs, the most common of which is vancomycin. We present a case of a 45-year-old woman who was diagnosed with vancomycin-induced LABD by the presence of a characteristic linear band of IgA along the basement membrane zone on direct immunofluorescence microscopy. Our patient showed complete recovery after a 2-week period during which vancomycin administration was discontinued.

A Case of Linear IgA Bullous Dermatosis Probably Caused by Captopril / 대한피부과학회지

Linear IgA bullous dermatosis (LABD) is a rare autoimmune subepidermal bullous disease characterized by a linear pattern of IgA deposition along the basement membrane zone. The etiology of LABD is unknown but a minority of cases are drug-induced. Although vancomycin is the agent most commonly implicated in the pathogenesis of drug-induced LABD, association with other agents including phenytoin, piroxicam, captopril, diclofenac sodium and cyclosporine have also been reported. We describe a case of linear IgA bullous dermatosis which probably developed after captopril treatment in a 51-year-old male patient with infective endocarditis.

A case of Linear IgA Bullous Dermatosis Probably Caused by Vancomycin / 대한피부과학회지

Linear IgA bullous dermatosis (LABD) is an uncommon acquired autoimmune subepidermal bullous disease characterized by the linear deposition of IgA at the basement membrane zone. Although LABD is most often idiopathic, drug-induced LABD may, occur in rare occasions. We report a case of vancomycin-induced LABD in a 70-year-old woman with septic arthritis on the right knee.

A Case of Vancomycin-Induced Linear IgA Bullous Dermatosis / 대한피부과학회지

Linear IgA bullous dermatosis(LAD) is a subepidermal blistering disease characterized by linear IgA deposits in the basement membrane zone when visualized on direct immunofluorescence microscopy. Intravenous vancomycin has been the drug most implicated, and this disease has been termed vancomycin-induced LAD. Vancomycin-induced LAD tends to spontaneously resolve upon discontinuation of the offending drug. A 70-year-old male patient developed septic knee. Bacterial cultures from this lesion grew methicillin- resistant Staphylococcus aureus. He was treated with vancomycin. Eight days after vancomycin injection, painful bullae appeared on the trunk, feet, and genitalia. A skin biopsy showed a subepidermal blister with an infiltrate composed of neutrophils and eosinophils. Direct immunofluorescence of perilesional skin showed linear IgA deposition at dermo-epidermal junction. The vancomycin was stopped; teicoplanin was substituted. He was treated with dapsone. The use of vancomycin is increasing in Korea and therefore it is important for dermatologist to be aware of the association with LAD.

A case of drug - induced linear IgA bullous dermatosis / 천식및알레르기

Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal blistering disease characterized on direct immunofluorescence of perilesional skin by the linear deposition of IgA at the basement membrane zone. The pathogenesis of LABD is not known, but rar ely some cases of LABD have been reported in association with drug exposure. We report a case of drug-induced LABD showing linear deposits of IgA and IgG at the basement membrane zone on direct immunofluorescence of perilesional skin as well as typical clinical and histologic characteristics, although the causative agent could not be specified.