Épidermolyse bulleuse acquise

Caux, F.

doi:10.1016/j.lpm.2010.04.021

Cited by 5 publications

(2 citation statements)

References 31 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This localization is distinct from the deposits in other AIBD [196–198]. Immunoelectron microscopy is considered the gold standard for EBA diagnosis.…”

Section: Diagnosis Of Ebamentioning

confidence: 98%

Clinical Presentation, Pathogenesis, Diagnosis, and Treatment of Epidermolysis Bullosa Acquisita

Ludwig

2013

ISRN Dermatology

124

View full text Add to dashboard Cite

Epidermolysis bullosa acquisita (EBA) is a chronic mucocutaneous autoimmune skin blistering disease. The pathogenic relevance of autoantibodies targeting type VII collagen (COL7) has been well-documented. Therefore, EBA is a prototypical autoimmune disease with a well-characterized pathogenic relevance of autoantibody binding to the target antigen. EBA is a rare disease with an incidence of 0.2 new cases per million and per year. The current treatment of EBA relies on general immunosuppressive therapy, which does not lead to remission in all cases. Therefore, there is a high, so far unmet medical need for the development of novel therapeutic options. During the last 10 years, several novel in vitro and in vivo models of EBA have been established. These models demonstrated a critical role of the genetic background, T cells, and cytokines for mediating the loss of tolerance towards COL7. Neutrophils, complement activation, Fc gamma receptor engagement, cytokines, several molecules involved in cell signaling, release of reactive oxygen species, and matrix metalloproteinases are crucial for autoantibody-induced tissue injury in EBA. Based on this growing understanding of the diseases' pathogenesis, several potential novel therapeutic targets have emerged. In this review, the clinical presentation, pathogenesis, diagnosis, and current treatment options for EBA are discussed in detail.

show abstract

“…This localization is distinct from the deposits in other AIBD [196–198]. Immunoelectron microscopy is considered the gold standard for EBA diagnosis.…”

Section: Diagnosis Of Ebamentioning

confidence: 98%

Clinical Presentation, Pathogenesis, Diagnosis, and Treatment of Epidermolysis Bullosa Acquisita

Ludwig

2013

ISRN Dermatology

124

View full text Add to dashboard Cite

show abstract

“…EBA is a chronic, autoimmune, subepidermal bullous disease with clinical features similar to the genetic form of dystrophic epidermolysis bullosa [120][121][122][123]. EBA has been differentiated from other bullous diseases on the basis of distinctive clinical and histologic and immunodermatologic features, establishing diagnostic criteria for the disease.…”

Section: Epidermolysis Bullosa Acquisita (Eba)mentioning

confidence: 99%

Autoimmune basement membrane and subepidermal blistering diseases

Velez¹,

Vasquez-Hincapie²,

Howard³

2013

Our Dermatol Online

View full text Add to dashboard Cite

Autoimmune mucocutaneous blistering diseases (ABDs) represent a group of conditions that manifest with blisters on the skin and/or mucous membranes. Bullous pemphigoid (BP) is the most common autoimmune mucocutaneous blistering disease. In BP, the location of the blisters is subepidermal and the oral involvement is rare. Variants of BP have been described, including pemphigoid vegetans; however, this disease is not completely characterized. The majority of ABDs have blisters and/or vesicles, that are often pruritic, and manifest autoantibodies to diverse proteins. These proteins include 1) hemidesmosomal plaque proteins(ie, BP230, plectins), 2) transmembrane proteins such as BP180 and α6β4-integrin, which are connected via laminin 332 to type VII collagen and 3) currently uncharacterized 105 kDa and 200 kDa molecules. Other ABDs include drug-induced linear IgA disease, bullous systemic lupus erythematosus (BSLE), dermatitis herpetiformis (DH), cicatricial pemphigoid (CP; also termed mucous membrane pemphigoid), lichen planus pemphigoides (LPP), pemphigoid gestationis (PG), herpes gestationis(HG), chronic bullous dermatosis of childhood (CBDC) and the localized forms of CP, such as Brunsting-Perry pemphigoid. The diagnosis of ABDs requires clinical data; skin biopsies (in 10% buffered formalin) for hematoxylin and eosin (H&E) examination and skin biopsies(in Michel's transport medium) for direct immunofluorescence (DIF). In many ABDs, the histopathologic findings demonstrate a subepidermal vesicle or bulla with a luminal inflammatory infiltrate of neutrophils, eosinophils and/or lymphocytes. In many ABDs, an extensive perivascular and interstitial inflammatory infiltrate is also noted subjacent to the blister in the upper dermis. Normal skin adjacent to an ABD plaque is often excellent for DIF results. Many ABD biopsies reveal autoantibody deposition at the lesional basement membrane zone (BMZ); IgG, IgM, IgA, other immunoglobulins, complement components and fibrinogen may be detected. Indirect immunofluorescence (IIF) yields antibody titer data; the titers usually correlate with disease activity and with ELISA. Linear epitopes are commonly studied by using an immunoblotting (IB) assay. Topical and systemic corticosteroids remain as mainstays of therapy in ABDs; however, multiple other immunosupressors and/or "steroid sparing agents" such as azathioprine have been demonstrated to be of therapeutic value. In the IgA mediated dermatoses, dapsone is often helpful; in addition, liver and blood testing (including G6PD levels) is indicated. The prognosis depends on each case; rapid diagnosis avoids complications and assists in maintaining a good quality of life for each patient.

show abstract

Generalisierte inflammatorische Epidermolysis bullosa acquisita bei initialem Virusexanthemverdacht

et al. 2014

View full text Add to dashboard Cite

A 67-year-old man presented with a maculopapular exanthem which started over the major joints. Within a few days, it spread to the entire skin surface and was accompanied by blisters as well as changes of the oral mucosa. The histological examination revealed subepidermal blistering. Targeted laboratory examinations detected serum antibodies against the epidermal basement membrane and autoantibodies against type VII collagen. The findings supported the diagnosis of a generalized inflammatory epidermolysis bullosa acquisita with mucosal involvement.

show abstract

Épidermolyse bulleuse acquise

Cited by 5 publications

References 31 publications

Clinical Presentation, Pathogenesis, Diagnosis, and Treatment of Epidermolysis Bullosa Acquisita

Clinical Presentation, Pathogenesis, Diagnosis, and Treatment of Epidermolysis Bullosa Acquisita

Autoimmune basement membrane and subepidermal blistering diseases

Generalisierte inflammatorische Epidermolysis bullosa acquisita bei initialem Virusexanthemverdacht

Contact Info

Product

Resources

About