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Pemphigus Vulgaris

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European Handbook of Dermatological Treatments

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Abstract

Pemphigus vulgaris is a life-threatening autoimmune disease caused by auto-antibodies against desmoglein 1 and 3. It arises in genetically predisposed individuals and is diagnosed based on clinical presentation, histopathology, direct immunofluorescence microscopy, and serological tests. The mainstay of treatment is systemic corticosteroids often combined with an adjuvant immunosuppressant, including mycophenolate mofetil, azathioprine, and rituximab. Recently, rituximab combined to a short course of oral corticosteroids has been shown to be highly effective in inducing complete remission as a first-line treatment compared to a conventional course of systemic corticosteroids. Rituximab was approved by the European Medicines Agency and the Food and Drug Administration as a first-line treatment for moderate to severe pemphigus. In more recalcitrant or severe diseases, the use of pulse intravenous methylprednisolone, high-dose intravenous immunoglobulins, or immunoadsorption may be considered.

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Abbreviations

aAbs:

Auto-antibodies

AZA:

Azathioprine

CBC:

Complete blood count

CP:

Cyclophosphamide

CR:

Complete remission

CS:

Corticosteroids

DIF:

Direct immunofluorescence

Dsg:

Desmoglein

ELISA:

Enzyme-linked immunosorbent assay

IA:

Immunoadsorption

IIF:

Indirect immunofluorescence

IL:

Intralesional

IV:

Intravenous

IVIg:

Intravenous immunoglobulins

MMF:

Mycophenolate mofetil

PDAI:

Pemphigus disease area index

PV:

Pemphigus vulgaris

RA:

Rheumatoid arthritis

RCT:

Randomized controlled trial

RTX:

Rituximab

TPMT:

Thiopurine methyltransferase

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Author information

Authors and Affiliations

  1. Department of Dermatology, Hôpital du Sacré-Coeur-de-Montréal, Montréal, QC, Canada

    Alexandre Lemieux

  2. Centre de référence des maladies bulleuses auto-immunes, Department of Dermatology, Centre Hospitalier Universitaire de Rouen, Rouen, France

    Pascal Joly

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  1. Alexandre Lemieux
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  2. Pascal Joly
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Correspondence to Alexandre Lemieux .

Editor information

Editors and Affiliations

  1. Andreas Syggros Hospital, Athens State University, Athens, Greece

    Andreas D. Katsambas

  2. Research Center CSRMR, Marconi University, Rome, Roma, Italy

    Torello M. Lotti

  3. Andreas Syggros Hospital, Athens State University, Athens, Greece

    Clio Dessinioti

  4. Dermatoloy Unit, Livorno Hospital, Livorno, Italy

    Angelo Massimiliano D'Erme

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Lemieux, A., Joly, P. (2023). Pemphigus Vulgaris. In: Katsambas, A.D., Lotti, T.M., Dessinioti, C., D'Erme, A.M. (eds) European Handbook of Dermatological Treatments. Springer, Cham. https://doi.org/10.1007/978-3-031-15130-9_69

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  • DOI: https://doi.org/10.1007/978-3-031-15130-9_69

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