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Comparison of Guidelines for Management of Pemphigus: a Review of Systemic Corticosteroids, Rituximab, and Other Immunosuppressive Therapies

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Abstract

Pemphigus is a severe autoimmune bullous dermatosis that affects the skin and/or mucosa, and it may be life-threatening without proper treatment. The guidelines and/or consensus statements for treatment vary widely between groups. We selected 6 guidelines and consensus statements established by different associations about the management of pemphigus vulgaris (PV) and/or pemphigus foliaceus (PF) to review, compare, and contrast the similarities and differences of these recommendations and provide optimal management suggestions to physicians. Corticosteroids remain a first-line therapy for pemphigus, but there are many differences in initial dose, tapering schedule, and management of relapse between different guidelines. Rituximab is a monoclonal antibody targeting CD20-positive B lymphocytes that is approved as a first-line therapy in moderate-to-severe pemphigus. Immunosuppressive agents, such as azathioprine (AZA) and mycophenolate mofetil (MMF), are also widely used as corticosteroid-sparing drugs, but the adjuvant applications and dosage regimens of different recommendations are not standardized. We attribute these differences to the clinical scoring adopted, the standards for disease severity evaluation, the publication year of each guideline, and local and regional healthcare differences.

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Abbreviations

ABSIS:

Autoimmune Bullous Skin Intensity and Severity Score

ASMSG:

Association of the Scientific Medical Societies in Germany

AZA:

Azathioprine

BAD:

British Association of Dermatologists

BSA:

Body surface area

BSD:

Brazilian Society of Dermatology

CS:

Corticosteroids

CsA:

Cyclosporine A

CTX:

Cyclophosphamide

DBC:

Dermatology Branch of China International Exchange and Promotion Association for Medical and Healthcare

Dsg:

Desmoglein

DXM:

Dexamethasone

EADV:

European Academy of Dermatology and Venereology

EDF:

European Dermatology Forum

FDA:

US Food and Drug Administration

FSD:

French Society of Dermatology

IA:

Immunoadsorption

IL:

Interleukin

IVIG:

Intravenous immunoglobulin

JAK:

Tyrosine kinases of the Janus family or Janus kinases

MP:

Methylprednisolone

MMF:

Mycophenolate mofetil

MPA:

Mycophenolic acid

MTX:

Methotrexate

PDAI:

Pemphigus Disease and Area Index

PDN:

Prednisone

PE:

Plasma exchange

PF:

Pemphigus foliaceus

PSL:

Prednisolone

PV:

Pemphigus vulgaris

RTX:

Rituximab

SCS:

Systematic corticosteroids

SCT:

Stem cell transplantation

TCS:

Topical corticosteroids

TPMT:

Thiopurine methyltransferase

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Funding

National Natural Science Foundation of China, Grant/Award Numbers: 81730085, 81773321.

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Author notes

  1. Haiqin Zhu and Meng Pan contributed equally to this work.

Authors and Affiliations

  1. Department of Dermatology, Rui Jin Hospital, Shanghai Jiao Tong University School of Medicine, 197 Rui Jin Er Road, Shanghai, 200025, China

    Wenzhe Zhao, Jingying Wang, Haiqin Zhu & Meng Pan

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  3. Haiqin Zhu
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  4. Meng Pan
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Zhao, W., Wang, J., Zhu, H. et al. Comparison of Guidelines for Management of Pemphigus: a Review of Systemic Corticosteroids, Rituximab, and Other Immunosuppressive Therapies. Clinic Rev Allerg Immunol 61, 351–362 (2021). https://doi.org/10.1007/s12016-021-08882-1

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