linear iga bullous dermatosis

Linear IgA bullous dermatosis (LABD) is characterized by presence of multiple IgA autoantibodies, and a comparatively lesser number of IgG antibodies, directed against different hemidesmosomal antigens. The main autoantigens are LAD-1, LABD-97, BP180 and BP230, type VII collagen and laminin 332. We retrospectively studied the serology of 54 Italian patients with LABD using enzyme-linked immunosorbent assay (ELISA), immunoblotting assay, and indirect immunofluorescence on monkey oesophagus and salt-split skin...

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Linear IgA dermatosis (LAD) is a rare autoimmune disorder in children. A 9-year-old boy was presented with blisters on the intact skin (face, body, arms, hands, soles, perigenital and perianal area) after amoxicillin treatment. Systemic corticosteroids and dapsone treatment for 6 weeks was successful. Clinical and immunofluorescence examinations are most important for differentiation of LAD and other drug-induced bullous dermatoses. They enable an early introduction of proper therapy.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

Vancomycin is a widely used antibiotic in hemodialysis patients. The main complications include renal toxicity and skin lesions. Herein, we report the case of a 29-year-old hemodialysis patient who presented a bullous pruriginous dermatosis after vancomycin treatment. A skin biopsy revealed a linear IgA bullous dermatosis (LABD). This is a rare form of dermatosis and is either idiopathic or more likely vancomycin-induced. Similarities in the molecular structure of vancomycin and epidermal basement membrane glycoproteins could explain the auto-immune response...

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive form of peripheral T-cell lymphoma, characterised by systemic symptoms, diffuse lymphadenopathy, hepatosplenomegaly and immunodysregulation. Half of AITL is associated with cutaneous symptoms, but only few cases with bullous eruption have been described. Association with a linear IgA dermatosis is extremely rare. Linear IgA dermatosis can be idiopathic, or linked with drug intake or neoplastic disorders. Some cases of linear IgA dermatosis presenting as toxic epidermal necrolysis (TEN) have been described, most of them being drug induced...

Linear immunoglobulin A (IgA) bullous dermatosis, also known as linear IgA disease, is a rare disorder with an incidence of about 0.5 to 2.3 cases per million individuals per year. In most of the cases, the cause is unknown; however, 50% of the cases are drug-induced. The disease has bimodal age predilection and occurs in children up to the age of 10 years and in adults usually after the age of 60 years. Common drugs that have been known to cause this disease include vancomycin, lithium, amiodarone, captopril, and some of the nonsteroidal anti-inflammatory agents...

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A 68-year-old woman was referred to the unit of dermatology for a large basal cell carcinoma on the chin. She was treated with imiquimod cream 5%, and 4 weeks after she developed isolated and grouped tense serum-filled vesicles and bullae on lips, nose, scalp, ankles and lumbar area, and then expanded to the whole body. Histological examination was consistent with a subepidermal bullous dermatosis. Moreover, direct immunofluorescence showed linear deposition of IgA at the basement membrane zone supporting the diagnosis of linear IgA bullous dermatosis (LABD)...

We report the case of a 59-year-old Japanese woman who developed linear IgA bullous dermatosis during treatment for ulcerative colitis that manifested as pruritic vesicles with erythema on the trunk and scalp. Histopathological examination revealed subepidermal bulla with neutrophil and eosinophil infiltration in the upper dermis. Direct immunofluorescence revealed linear IgA deposits at the basement membrane zone, and indirect immunofluorescence using split skin revealed IgA reaction to the epidermal side (lamina lucida type)...

Lichen planus pemphigoides (LPP) is a very rare autoimmune sub-epidermal blistering disease associated with lichenoid skin changes. Initially thought to be a mere variant of more common inflammatory dermatoses, particularly Bullous Pemphigoid (BP) or Lichen Planus (LP), a growing body of evidence suggests that it is a disease entity in its own right. In common with a range of autoimmune blistering diseases, including BP, pemphigoid gestationis (PG), mucous membrane pemphigoid (MMP) and linear IgA dermatosis (LAD), a key feature of the disease is the development of autoantibodies against type XVII collagen (COL17)...

We present four clinicopathological correlated cases of young patients with cryothermic dermatitis artefacta. They were initially misdiagnosed as primary bullous dermatoses or fixed drug eruptions. Cryothermic dermatitis artefacta can imitate authentic dermatoses such as linear IgA bullous dermatosis, herpes virus infection, bullous pemphigoid or fixed drug eruption. It should be considered as differential diagnosis in uncommon cases of recurrent bullae in adolescent and young adult patients. We summarize helpful clinical and histopathological criteria for correct diagnosis and therewith causative treatment...

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...

BACKGROUND: Linear IgA bullous dermatosis (LABD) is a rare autoimmune subepithelial vesiculobullous disease due to IgA autoantibodies directed against different antigens of the basement membrane zone (BMZ) of the skin and/or mucosae. It affects mainly preschool-aged children and adults, with only few studies on large series. The aim of this study was to assess possible differences between adults and children regarding clinical presentation, immunopathologic features, management and course of the disease...

We report an uncommon presentation of bullous dermatosis by linear IgA. There are few cases reported in the literature with this form of presentation starting with mucosal lesions and then evolving into a similar bullous pemphigoid pattern. In addition, we emphasize the importance of direct immunofluorescence for the definitive diagnosis.

Linear IgA bullous dermatosis is an acquired subepidermal immunoglobulin-mediated vesiculobullous disease. In this retrospective, observational, descriptive study, we describe the clinical characteristics, treatments, and outcomes of 17 patients with linear IgA bullous dermatosis. Two children had been vaccinated 2 weeks before the onset of symptoms, 2 had had bronco-obstructive respiratory symptoms, and 1 had received intravenous antibiotic therapy. We also observed an association with autoimmune hepatitis in one patient and alopecia areata in another...

An 8-month-old child was admitted to a paediatric intensive care unit in Guinea-Bissau with severe blistering dermatosis. He was treated with broad spectrum antibiotics and dressings, without improvement. After 2 weeks, linear IgA bullous dermatosis was suspected. Owing to lack of dapsone, the child was treated with prednisolone and improved. To avoid corticosteroids side effects, 2 months after starting prednisolone we switched to colchicine, but the boy's condition worsened for reasons of poor adherence, requiring intravenous corticosteroids and antibiotics...