Mucous membranesBullous dermatosisDrug-induced linear IgA dermatosisCicatricialEpidermolysis bullosaAntigensSubepidermalAntibodiesAutoimmune blisteriLesionsAntibodyPemphigusDepositionAntigenMucosalCollagenDiseasesLinearSkinPatientBullous pemphigoidAutoimmuneEpidermolysis bullosa acquisitaImmunoglobulinPemphigoidAlpha3OcularImmunofluorescenceCells
Mucous membranes7
- A group of autoimmune blistering diseases affecting primarily the mucous membranes is termed mucous membrane pemphigoid (MMP) (also termed cicatricial pemphigoid). (medscape.com)
- Introduction: Autoimmune bullous diseases (AIBDs) are a group of rare cutaneous disorders affecting cornified skin and mucous membranes. (bvsalud.org)
- Pemphigoid is a group of rare autoimmune blistering diseases of the skin, and mucous membranes. (wikipedia.org)
- Bullous pemphigoid is a rare and chronic autoimmune disorder characterised by large sub-epidermal blisters called bullae, that predominantly involves the skin and less commonly the mucous membranes. (wikipedia.org)
- Primary lesions of small and large blisters, known as vesicles and bullae, are found on the skin and sometimes on the mucous membranes. (wikipedia.org)
- A rare autoimmune bullous skin disease characterized clinically by blistering of the mucous membranes followed by scarring, and immunologically characterized by IgG, IgA and/or C3 deposits on the epidermal basement membrane. (nih.gov)
- The disease principally involves the oral mucosa, but may also affect ocular, pharyngolaryngeal, genital, and esophageal mucous membranes. (nih.gov)
Bullous dermatosis10
- Three cases of linear IgA/IgG bullous dermatosis showing IgA and IgG reactivity with multiple antigens, particularly laminin-332. (medscape.com)
- Zenke Y, Nakano T, Eto H, Koga H, Hashimoto T. A case of vancomycin-associated linear IgA bullous dermatosis and IgA antibodies to the a3 subunit of laminin-332. (medscape.com)
- Nartker N, Kudlak N, Crowe D. Linear IgA bullous dermatosis protracted by vancomycin-loaded bone cement. (medscape.com)
- Drug-induced linear IgA bullous dermatosis in a patient with a vancomycin-impregnated cement spacer. (medscape.com)
- Sanke S, Kumar A, Chander R. Linear IgA bullous dermatosis induced by diclofenac sodium. (medscape.com)
- Hoffmann J, Hadaschik E, Enk A, Stremmel W, Gauss A. Linear IgA Bullous Dermatosis Secondary to Infliximab Therapy in a Patient with Ulcerative Colitis. (medscape.com)
- Children and adults are affected, with disease of the former historically referred to as chronic bullous dermatosis of childhood. (medscape.com)
- Some patients possess IgA and IgG antibodies toward laminin 332 in what Zone et al described as linear IgA/IgG bullous dermatosis. (medscape.com)
- This article discusses the oral manifestations of several well-characterized autoimmune blistering diseases, including pemphigus vulgaris, bullous pemphigoid, linear immunoglobulin A (IgA) bullous dermatosis, and paraneoplastic pemphigus (PNP). (medscape.com)
- [ 5 ] linear IgA bullous dermatosis (dogs), epidermolysis bullosa acquisita (dogs), and bullous systemic lupus erythematosus (1 dog). (medscape.com)
Drug-induced linear IgA dermatosis2
- [ 37 ] Particularly severe cases of drug-induced linear IgA dermatosis respond to a short course of oral corticosteroids. (medscape.com)
- The etiology underlying drug-induced linear IgA dermatosis remains elusive but is speculated to be caused by an immune response towards a drug-derived hapten-protein antigen. (medscape.com)
Cicatricial2
- Laminin-332 polypeptides are targeted by auto-antibodies in human patients with mucous membrane (cicatricial) pemphigoid or, more rarely, subepidermal vesicular diseases that resemble epidermolysis bullosa acquisita (EBA) or bullous pemphigoid (BP). (morulaivf.com)
- There are several types: Gestational pemphigoid (PG) (formerly called Herpes gestationis) Bullous pemphigoid (BP) Rarely affects the mouth Mucous membrane pemphigoid (MMP) or (cicatricial pemphigoid), (No skin involvement) Bullous and mucous membrane pemphigoid usually affect persons who are over age 60. (wikipedia.org)
Epidermolysis bullosa3
- The names 'acquired junctional epidermolysis bullosa', 'anti-laminin-332 mucous membrane pemphigoid (MMP)' and 'mixed auto-immune subepidermal blistering dermatosis' are proposed for dogs with clinical signs reminiscent of EBA, MMP or BP respectively. (morulaivf.com)
- The evaluation included the diagnoses of pemphigus vulgaris (PV), pemphigus foliaceus (PF), bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), linear IgA dermatosis (LAD), epidermolysis bullosa acquisita (EBA), and dermatitis herpetiformis (DH). (bvsalud.org)
- A Review Comparing International Guidelines for the Management of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid, and Epidermolysis Bullosa Acquisita. (nih.gov)
Antigens2
- The authors suggest that an IgA-specific immune response may occur against bullous pemphigoid antigens in linear IgA dermatosis. (medscape.com)
- In the immunologic component, autoantibodies act against the hemidesmosomal bullous pemphigoid antigens BP230 (BPAg1) and BP 180(BPAg2 or type XVII collagen) which are located at the lamina lucida of the basement membrane zone. (wikipedia.org)
Subepidermal1
- Linear immunoglobulin A (IgA) dermatosis (LAD) is an autoimmune subepidermal vesiculobullous disease that may be idiopathic or drug-induced. (medscape.com)
Antibodies9
- Children with linear IgA dermatosis may demonstrate circulating anti-BMZ antibodies more frequently than adults. (medscape.com)
- Antigenic specificity of antibodies from patients with linear basement membrane deposition of IgA. (medscape.com)
- The best-characterized antigen is a 97-kd protein extracted from human epidermis that binds IgA antibodies from sera of patients with linear IgA dermatosis. (medscape.com)
- this antigen is recognized by circulating antibodies in some patients with linear IgA dermatosis, but it has not been further characterized. (medscape.com)
- [ 4 ] Antilaminin 332 antibodies are typically seen in malignancy-associated mucous membrane pemphigoid (MMP). (medscape.com)
- Polyclonal and monoclonal antibodies against chicken gizzard 5 '-nucleotidase inhibit the spreading process of chicken embryonic fibroblasts on laminin substratum. (morulaivf.com)
- Polyclonal and monoclonal antibodies raised against chicken gizzard 5′-nucleotidase were tested in adhesion assays of embryonic chicken fibroblasts (CEF) for their ability to interfere with the adhesion process of these cells on either laminin or fibronectin substrata. (morulaivf.com)
- The initial attachment process of CEF on fibronectin and laminin substrata was not influenced by preincubating these cells with antibodies against chicken gizzard 5′-nucleotidase. (morulaivf.com)
- In all nine dogs, IgG auto-antibodies detected purified human laminin-332 by immunoblotting. (morulaivf.com)
Autoimmune blisteri2
- These observations establish laminin-332 as a novel basement membrane antigen in dogs with autoimmune blistering diseases with variable clinical phenotypes. (morulaivf.com)
- Immune disturbance as well as destruction of basal epidermal cells and base membrane by GVHD may result in the induction of autoimmune blistering diseases with unusual clinical and laboratory manifestations. (morulaivf.com)
Lesions3
- In cases of linear IgA dermatosis induced by vancomycin, new lesions stop forming within approximately 2 weeks of withdrawal. (medscape.com)
- Bullous lesions on the genital area in a child with linear IgA dermatosis. (medscape.com)
- Bullous lesions of the palmar surface in an elderly man with vancomycin-induced linear immunoglobulin A (IgA) dermatosis. (medscape.com)
Antibody3
- Approximately 50% of patients with linear IgA dermatosis have detectable circulating antibody that binds to the BMZ. (medscape.com)
- Prednisone stabilizes lysosomal membranes and suppresses lymphocyte and antibody production. (medscape.com)
- In vitro assays demonstrated a competition of laminin and this monoclonal antibody for the binding site on purified 5′-nucleotidase. (morulaivf.com)
Pemphigus1
- Whereas the pemphigus subset of diseases is mediated by autoantibodies that target the extracellular skin components that link one epidermal cell to another, the pemphigoid subset is mediated by autoantibodies that target the extracellular skin components that link the skin basement membrane components either to the lowermost layer of epidermal cells or to the dermal components. (medscape.com)
Deposition2
- Linear IgA dermatosis is an autoimmune disease histopathologically characterized by the linear deposition of IgA at the basement membrane zone (BMZ). (medscape.com)
- Direct immunofluorescence revealed a linear deposition of IgG on the base membrane zone. (morulaivf.com)
Antigen2
- These results are provocative given that the 97-kd linear IgA dermatosis antigen may represent a portion of the extracellular domain of BPAg2. (medscape.com)
- Linear IgA dermatosis illustrates the importance of identifying the target antigen. (medscape.com)
Mucosal1
- It usually does not affect oral and other mucosal membranes. (medscape.com)
Collagen2
- Immunohistochemical staining of laminin 332 and collagen IV along the floor of bullae can aid in the diagnosis, although the finding is nonspecific and can be seen in other bullous dermatoses where the schism lies above the lamina densa. (medscape.com)
- Immunological investigations consisted of direct immunofluorescence (IF), indirect IF with intact and salt-split canine gingival, and salt-split normal or laminin-332-deficient human skin, immunoblotting with purified human laminin-332 and immunoblotting with recombinant NC1 domain of human collagen VII. (morulaivf.com)
Diseases1
- High incidence of autoimmune diseases and potential complications, including the development of lymphoma have been demonstrated in this dermatosis. (scielo.br)
Linear6
- Neutrophilic microabscesses in linear immunoglobulin A (IgA) dermatosis. (medscape.com)
- Large, randomized, placebo-controlled, double-blind studies have not been performed for the treatment of linear IgA dermatosis in children or adults. (medscape.com)
- These agents have been shown to be beneficial in the treatment of linear IgA dermatosis. (medscape.com)
- Although an antibiotic, tetracycline has proven effective in linear IgA dermatosis, either alone or in conjunction with niacinamide (2 g/d). (medscape.com)
- It is likely that some cases initially described were of bullous pemphigoid or linear IgA dermatosis bullosa, given the unavailability of better diagnostic tools at that period. (scielo.br)
- In 1969 Van der Meer, in turn, revealed the occurrence of granular IgA deposits in those same locations, which constituted a major milestone for the understanding of this disease and permitted for the first time its differentiation from linear IgA dermatosis bullosa. (scielo.br)
Skin1
- In five dogs, IgG variably recognized the basement membrane of laminin-332-deficient human skin (three dogs negative, two dogs positive). (morulaivf.com)
Patient1
- Costello first described, in 1940, the effectiveness of sulfapyridine in a patient with this dermatosis. (scielo.br)
Bullous pemphigoid7
- 2. A child with antibodies targeting both linear IgA bullous dermatosis and bullous pemphigoid antigens. (nih.gov)
- 5. Bullous pemphigoid positive for anti-BP180 and anti-laminin 5 antibodies in a patient with graft-vs-host disease. (nih.gov)
- 11. 97 kDa linear IgA bullous dermatosis antigen localizes in the lamina lucida between the NC16A and carboxyl terminal domains of the 180 kDa bullous pemphigoid antigen. (nih.gov)
- In the immunologic component, autoantibodies act against the hemidesmosomal bullous pemphigoid antigens BP230 (BPAg1) and BP 180(BPAg2 or type XVII collagen) which are located at the lamina lucida of the basement membrane zone. (wikipedia.org)
- This article discusses the oral manifestations of several well-characterized autoimmune blistering diseases, including pemphigus vulgaris, bullous pemphigoid, linear immunoglobulin A (IgA) bullous dermatosis, and paraneoplastic pemphigus (PNP). (medscape.com)
- A Review Comparing International Guidelines for the Management of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid, and Epidermolysis Bullosa Acquisita. (nih.gov)
- Bullous Pemphigoid and Other Pemphigoid Dermatoses. (nih.gov)
Autoimmune3
- 13. Autoimmune responses in patients with linear IgA bullous dermatosis: both autoantibodies and T lymphocytes recognize the NC16A domain of the BP180 molecule. (nih.gov)
- These observations establish laminin-332 as a novel basement membrane antigen in dogs with autoimmune blistering diseases with variable clinical phenotypes. (morulaivf.com)
- Immune disturbance as well as destruction of basal epidermal cells and base membrane by GVHD may result in the induction of autoimmune blistering diseases with unusual clinical and laboratory manifestations. (morulaivf.com)
Epidermolysis bullosa acquisita1
- [ 5 ] linear IgA bullous dermatosis (dogs), epidermolysis bullosa acquisita (dogs), and bullous systemic lupus erythematosus (1 dog). (medscape.com)
Immunoglobulin2
- Neutrophilic microabscesses in linear immunoglobulin A (IgA) dermatosis. (medscape.com)
- 18. A Case of Possible Concurrence of Dermatitis Herpetiformis and Linear Immunoglobulin A / Immunoglobulin G Bullous Dermatosis. (nih.gov)
Pemphigoid3
- The first international consensus on mucous membrane pemphigoid was published in 2002. (dianneelizabeth.com)
- Whereas the pemphigus subset of diseases is mediated by autoantibodies that target the extracellular skin components that link one epidermal cell to another, the pemphigoid subset is mediated by autoantibodies that target the extracellular skin components that link the skin basement membrane components either to the lowermost layer of epidermal cells or to the dermal components. (medscape.com)
- European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part II. (nih.gov)
Alpha31
- Indirect immunofluorescence using skin substrate lacking an epidermal basement membrane molecule, direct immunoelectron microscopy, immunoblot, and immunoprecipitation studies revealed the simultaneous presence of circulating IgG autoantibodies against type VII collagen and laminin alpha3. (nih.gov)
Ocular1
- Patients with linear IgA dermatosis can have changes, such as fine scarring, in the absence of ocular complaints. (medscape.com)
Immunofluorescence2
- Immunological investigations consisted of direct immunofluorescence (IF), indirect IF with intact and salt-split canine gingival, and salt-split normal or laminin-332-deficient human skin, immunoblotting with purified human laminin-332 and immunoblotting with recombinant NC1 domain of human collagen VII. (morulaivf.com)
- Direct immunofluorescence revealed a linear deposition of IgG on the base membrane zone. (morulaivf.com)
Cells2
- However, the subsequent spreading process of these cells was found to be inhibited for at least 2 h on a laminin substratum. (morulaivf.com)
- Spreading-arrested and rounded CEF do not develop prominent intracellular stress-fibers like control cells, instead they seem to concentrate their available actin in areas of presumptive initial contact with the laminin substratum. (morulaivf.com)