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.

Enfermedad de Sneddon-Wilkinson / Sneddon-Wilkinson disease

Introducción: la dermatosis pustulosa subcórnea o enfermedad de Sneddon-Wilkinson, es una enfermedad poco común, que se distingue por pústulas flácidas, recurrentes y coalescentes; su mecanismo etiológico no está del todo dilucidado, pero se relaciona con la presencia de enfermedades neoplásicas o inmunológicas. Presentación del caso: se presenta el caso de un niño de 4 años de edad, que acude a consulta de Dermatología del Hospital Andino de Chimborazo, con manifestaciones clínicas que permiten hacer el diagnóstico de dermatosis pustulosa subcórnea. Conclusiones: la enfermedad de Sneddon-Wilkinson es una afección rara que se presenta frecuentemente en pacientes femeninas de 40 años y más. Se caracteriza por una amplia diversidad de manifestaciones cutáneas que dificultan el pronóstico y la evolución del paciente. Esta enfermedad, cuando se presenta en niños, puede confundirse con otras afecciones que cursan con exantemas cutáneos(AU)

Introduction: subcorneal pustular dermatosis or Sneddon-Wilkinson disease is a rare illness characterized by recurrent, coalescent and flaccid pustules; its etiological mechanism is not totally known, but it is related to neoplastic or immunological diseases. Case presentation: a four-year old child who went to the dermatology service of Hospital Andino de Chimborazo. He presented with clinical manifestations that allow making the diagnosis of subcorneal pustular dermatosis. Conclusions: Sneddon-Wilkinson disease is a rare illness that frequently occurs in 40 years-old and older women. It has a wide range of skin manifestations that makes prognosis and recovery of patient difficult. When it appears in children, it may create confusion with other illnesses having skin exanthemas(AU)

Dermatosis ampollar por inmunoglobulina A lineal: Reporte de dos casos / Linear immunoglobulin A bullous dermatosis: Two cases report

La dermatosis ampollar por inmunoglobulina A lineal es una rara enfermedad, generalmente autolimitada, que afecta a niños de 4,5 años (edad media), con una incidencia de 0,52,3 casos/millón de habitantes/año. Es, tras la dermatitis herpetiforme, la enfermedad ampollar pediátrica más frecuente. Ocurre en brotes con lesión patognomónica en collar de perlas y afecta preferentemente la zona genital y peribucal. Su diagnóstico se basa en una alta sospecha clínica y en la biopsia de piel con observación de ampollas subepidérmicas y depósito lineal de inmunoglobulina A en inmunofluorescencia directa. Frecuentemente, el diagnóstico es tardío debido al desconocimiento de esta enfermedad.

Linear immunoglobulin A bullous dermatosis is a rare entity with frequent spontaneous resolution. It usually presents in children with average age of 4.5 years. Its incidence is about 0.5-2.3 cases/million individuals/year. It is, after dermatitis herpetiformis, the most frequent paediatric blister disorder. It usually appears in bouts with acute development of vesicles in strings of pearls; affecting the perioral area and genitalia. Diagnosis is based on the clinical signs and symptoms and biopsy of the skin with subepidermal blister and a linear band of immunoglobulin A in the direct immunofluorescence. Often, diagnosis is made late because of the unawareness of this disease.

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.

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.

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 necessidade da imunofluorescência direta no diagnóstico da dermatose bolhosa por IgA / The need for direct immunofluorescence in the diagnosis of IgA bullous dermatosis

A dermatose bolhosa por imunoglobulina da classe A linear (DbIgA) do adulto é uma doença autoimune rara caracterizada por formação de bolhas subepidérmicas e depósito linear de imunoglobulina da classe A (IgA) na zona da membrana basal (ZMB). Por possuir aspectos clínicos e histológicos semelhantes a outras dermatoses bolhosas, principalmente a dermatite herpetiforme e o penfigoide bolhoso, faz-se necessária a realização de imunofluorescência direta para confirmação diagnóstica. Apresenta-se então, neste artigo, relato de caso ilustrando essa necessidade.

Linear immunoglobulin A bullous dermatosis (DbIgA) of adults is a rare autoimmune disease characterized by subepidermal blistering and linear deposits of immunoglobulin A (IgA) in the basement membrane zone (BMZ). Owing to the fact it presents clinical and histological aspects similar to other bullous dermatosis, mainly dermatitis herpetiformis and bullous pemphigoid, direct immunofluorescence is required to confirm diagnosis. In this article, we describe a case that illustrates this need.

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.

Dermatosis por IgA lineal de la infancia. Reporte de un caso

La dermatosis por IgA lineal de la infancia es una enfermedad ampollar, auto inmune, adquirida, usualmente en la edad pre-escolar. Histológicamente se observa una ampolla subepidérmica y en la inmunofluorescencia directa, depósitos lineales de IgA. La respuesta al tratamiento es generalmente favorable. Presentamos una paciente femenina de 3 años de edad, con cuadro de cinco días de aparición de máculas eritematosas, pruriginosas en extremidades y tronco, sobre los cuales luego de una día aparecieron vesículas y ampollas. Se le realiza biopsia de piel e inmunofluorescencia directa las cuales confirman el diagnóstico de dermatosis de IgA lineal de la infancia. Se inicia tratamiento con corticoides tópicos y endovenosos, antibióticos y antihistamínicos demostrando mejoría de las lesiones y tendencia a la curación a las 3 semanas de evolución.

Linear IgA dermatosis of childhood is an autoimmune, bullous, acquired disease, predominantly in pre-school children. Histologically you should observe a sub epidermal bulla, the direct immunofluorescence shows linear deposits of IgA along the basement membrane. Generally there is a good response to treatment. We present the clinical case of a 3 years old girl, who had five days with erythematous, pruriginous, macule in her limbs and trunk, followed by the appearance of vesicles and bulla. We did a skin biopsy and direct immunofluorescence that confirmed the diagnosis of linear IgA dermatosis of childhood. We started treatment with topical and systemic steroids, antibiotics and antihistaminic, showing improvement of the lesion at 3 weeks.

Dermatosis IgA lineal: revisión y presentación de caso clínico / Linear IgA dermatosis: a case report and literature review

La Dermatosis IgA lineal es una enfermedad vesiculoampollar subepidérmica autoinmune caracterizada por anticuerpos IgA en la unión dermoepidérmica. Es una enfermedad poco frecuente, siendo la mayoría de los casos idiopáticos, pero con reporte de casos por medicamentos, infecciones virales, enfermedades autoinmunes y tumores malignos. Se presentará un caso clínico de Dermatosis IgA lineal causada por Diclofenaco.

Linear IgA dermatosis is an autoimmune subepidermal vesiculobullous disease characterized by IgA antibodies at the dermo-epidermal junction. It is an uncommon disease, with most cases idiopathic, but with case reports caused by drug, viral infections, autoimmune diseases and malignant tumors. A clinical case of Linear IgA dermatosis caused by diclofenac is presented.