Pemphigus and bullous pemphigoid

Nousari, Hossein C.; Anhalt, Grant J.

doi:10.1016/s0140-6736(99)03007-x

Cited by 167 publications

(195 citation statements)

References 88 publications

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“…*P < 0.05; **P < 0.001. (8). Subepidermal blistering in BP is induced by pathogenic anti-BP180 IgG and depends on complement activation, mast cell degranulation, and neutrophil infiltration (18)(19)(20).…”

Section: Figurementioning

confidence: 99%

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Complete FcRn dependence for intravenous Ig therapy in autoimmune skin blistering diseases

Zhao²,

Hilario-Vargas³

et al. 2005

Journal of Clinical Investigation

229

189

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Numerous mechanisms of action have been proposed for intravenous Ig (IVIG). In this study, we used IgG passive transfer murine models of bullous pemphigoid (BP), pemphigus foliaceus (PF), and pemphigus vulgaris (PV) to test the hypothesis that the effect of IVIG in autoantibody-mediated cutaneous bullous diseases is to accelerate the degradation of pathogenic IgG by saturation of the MHC-like Fc receptor neonatal Fc receptor (FcRn). BP, PF, and PV are organ-specific antibody-mediated diseases in which autoantibodies target the hemidesmosomal antigen BP180 and desmosomal antigens Dsg1 and Dsg3, respectively. Antibodies against BP180, Dsg1, and Dsg3, when injected into neonatal mice, induce the BP, PF, and PV disease phenotypes, respectively. We found that FcRn-deficient mice were resistant to experimental BP, PF, and PV. Circulating levels of pathogenic IgG in FcRndeficient mice were significantly reduced compared with those in WT mice. Administration of high-dose human IgG (HDIG) to WT mice also drastically reduced circulating pathogenic IgG levels and prevented blistering. In FcRn-deficient mice, no additional protective effect with HDIG was realized. These data demonstrate that the therapeutic efficacy of HDIG treatment in the pemphigus and pemphigoid models is dependent on FcRn. Thus, FcRn is a promising therapeutic target for treating such IgG-mediated autoimmune diseases.

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Section: Figurementioning

confidence: 99%

“…BP180-specific autoantibodies predominantly target epitopes located within the NC16A region of the ectodomain of the molecule (6,7). Pemphigus is characterized by intraepidermal blisters and epidermis-specific autoantibodies (8). The 2 major forms of the disease are pemphigus foliaceus (PF) and pemphigus vulgaris (PV).…”

Section: Introductionmentioning

confidence: 99%

Complete FcRn dependence for intravenous Ig therapy in autoimmune skin blistering diseases

Zhao²,

Hilario-Vargas³

et al. 2005

Journal of Clinical Investigation

229

189

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“…ullous pemphigoid (BP) is a disease of the elderly characterized by tense blisters and severe pruritus (1)(2)(3). Autoantibodies in BP are directed against two hemidesmosomal proteins, BP230 and BP180 (type XVII collagen) (4-7).…”

mentioning

confidence: 99%

Repetitive Immunization Breaks Tolerance to Type XVII Collagen and Leads to Bullous Pemphigoid in Mice

Hirose

Recke

Beckmann

et al. 2011

The Journal of Immunology

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Bullous pemphigoid (BP) is a subepidermal autoimmune blistering disease of the elderly associated with considerable morbidity and mortality. As unspecific immunosuppressants are still the mainstay of BP therapy, several animal models, based on the passive transfer of autoantibodies or immune cells, have been developed to obtain a better understanding of the pathogenesis of BP and evaluate novel therapeutic interventions. We describe in this study an experimental model inducing BP by immunization of immunocompetent mice with a recombinant form of the immunodominant 15th noncollagenous domain of murine BP180 (type XVII collagen). The homologous noncollagenous 16A domain of human BP180 has previously been identified as an immunodominant region in human BP. Immunization of female SJL/J mice with the murine peptide led to clinical disease within 14 wk in 56% of mice. In contrast, none of the other strains developed blisters despite the presence of autoantibodies. The clinical disease manifested for at least 8 wk without further manipulation. This novel immunization-induced model reflects key immunopathological characteristics of human BP, including binding of complement-fixing autoantibodies along the dermal–epidermal junction, elevated total IgE serum levels, and infiltration of skin lesions with eosinophilic granulocytes. The use of immunocompetent mice and the induction of sustained clinical disease not requiring additional interventions make this immunization-induced mouse model most suitable to further explore the pathogenesis of BP and novel therapeutic interventions for this and other autoantibody-mediated diseases.

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“…En los casos rebeldes o con el objetivo de reducir la dosis de corticoides se pueden asociar inmunosupresores como la azatioprina o ciclofosfamida 6,7 . Otros tratamientos utilizados son dapsona, micofenolato mofetil, o en casos muy rebeldes la plasmaféresis 8 . En aquellos pacientes con enfermedad leve o poco extensa se pueden utilizar corticoides tópicos, aunque hay estudios recientes que afirman que los corticoides administrados de forma tópica son tan efectivos como los orales en formas moderadas y severas con menores efectos secundarios 9 .…”

Section: Discussionunclassified

Un caso de lesiones ampollosas generalizadas

Moreno¹,

Pérez

Pernaut

2003

Medifam

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INTRODUCCIÓNLa aparición de lesiones ampollosas es un hecho relativamente frecuente que incluye desde patologías banales autolimitadas hasta procesos graves con elevada morbimortalidad. En algunos casos la ampolla es un signo añadido, pero existe un grupo de enfermedades entre las que figura el penfigoide ampolloso, en las cuales esta lesión constituye el verdadero sustrato patológico. CASO CLÍNICO

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Pemphigus and bullous pemphigoid

Cited by 167 publications

References 88 publications

Complete FcRn dependence for intravenous Ig therapy in autoimmune skin blistering diseases

Complete FcRn dependence for intravenous Ig therapy in autoimmune skin blistering diseases

Repetitive Immunization Breaks Tolerance to Type XVII Collagen and Leads to Bullous Pemphigoid in Mice

Un caso de lesiones ampollosas generalizadas

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