Pemphigus

Eming, Rüdiger

doi:10.1007/s00105-015-3656-3

Cited by 4 publications

(2 citation statements)

References 80 publications

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“…However, especially under long-time therapy, these medications cause many side effects including increased infection tendency, osteoporosis, low blood sugar, weight gain arterial hypertonia, cataracts, and glaucoma. These side effects can be reduced or delayed by combination therapy with other immune-suppressive agents or antibodies such as Rituximab, which depletes pathogenic B cells, to reduce the steroid dose ( 72 – 75 ). Current combination therapies reduce the mortality to about 5–10% with death mostly resulting from adverse drug side effects ( 76 ).…”

Section: Introductionmentioning

confidence: 99%

“…However, all these therapies have their own drawbacks, including the high costs ( 83 ), availability of donor IgG ( 84 ) and the need for regular long treatment sessions. Currently the treatment with corticosteroids in combination with immune suppressive agents and Rituximab is still the standard method ( 72 , 85 , 86 ). Furthermore, it is known that not all patients respond to these therapy concepts.…”

Section: Introductionmentioning

confidence: 99%

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Autoantibody-Specific Signalling in Pemphigus

Schmitt

Waschke

2021

Front. Med.

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Pemphigus is a severe autoimmune disease impairing barrier functions of epidermis and mucosa. Autoantibodies primarily target the desmosomal adhesion molecules desmoglein (Dsg) 1 and Dsg 3 and induce loss of desmosomal adhesion. Strikingly, autoantibody profiles in pemphigus correlate with clinical phenotypes. Mucosal-dominant pemphigus vulgaris (PV) is characterised by autoantibodies (PV-IgG) against Dsg3 whereas epidermal blistering in PV and pemphigus foliaceus (PF) is associated with autoantibodies against Dsg1. Therapy in pemphigus is evolving towards specific suppression of autoantibody formation and autoantibody depletion. Nevertheless, during the acute phase and relapses of the disease additional treatment options to stabilise desmosomes and thereby rescue keratinocyte adhesion would be beneficial. Therefore, the mechanisms by which autoantibodies interfere with adhesion of desmosomes need to be characterised in detail. Besides direct inhibition of Dsg adhesion, autoantibodies engage signalling pathways interfering with different steps of desmosome turn-over. With this respect, recent data indicate that autoantibodies induce separate signalling responses in keratinocytes via specific signalling complexes organised by Dsg1 and Dsg3 which transfer the signal of autoantibody binding into the cell. This hypothesis may also explain the different clinical pemphigus phenotypes.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Autoantibody-Specific Signalling in Pemphigus

Schmitt

Waschke

2021

Front. Med.

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Molekulare Diagnostik der blasenbildenden Autoimmundermatosen

Hoffmann

Hertl²,

Sitaru

2015

Hautarzt

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Bullous autoimmune diseases are organ-specific disorders characterized by an autoantibody-mediated blistering of skin and mucous membranes. The detection of tissue-bound and serum autoantibodies is prerequisite for the diagnosis of autoimmune blistering diseases. The individual entities of this group may be difficult to differentiate on clinical grounds alone. An accurate diagnosis is however important for prognosis and therapy. A preliminary diagnostic step includes direct and indirect immunofluorescence microscopy, which provide information about the binding pattern and isotype of autoantibodies and allow the diagnosis of the autoimmune blistering disease. Subsequent characterization of the molecular specificity of autoantibodies is necessary for the exact classification of autoimmune bullous dermatoses. The quantitative measurement of autoantibodies against structural proteins of the skin may be often used to assess disease severity at follow-up.

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Schleimhautbeteiligung bei blasenbildenden Autoimmunerkrankungen

Günther¹

2016

Hautarzt

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Autoimmune bullous diseases are characterized by intraepidermal or subepidermal autoantibody deposition that leads to blisters and secondary erosion. Mucous membranes are frequently affected in pemphigus vulgaris and always involved in cicatricial and mucosal pemphigoid. Mucosal lesions are detected less frequently in patients with bullous pemphigoid or epidermolysis bullosa acquisita. The diagnosis of autoimmune bullous disorders is based on determination of the subtype of autoantibodies bound in the skin and the clinical picture. Treatment is based on immunosuppression related to the type of disease and severity of the mucosal symptoms. Ocular involvement in mucosal pemphigoid and pemphigus vulgaris requires systemic treatment.

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Pemphigus

Cited by 4 publications

References 80 publications

Autoantibody-Specific Signalling in Pemphigus

Autoantibody-Specific Signalling in Pemphigus

Molekulare Diagnostik der blasenbildenden Autoimmundermatosen

Schleimhautbeteiligung bei blasenbildenden Autoimmunerkrankungen

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