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Pemphigus

  • Overview
  • Theory
  • Diagnosis
  • Management
  • Follow up
  • Resources
Last reviewed: 1 Oct 2025
Last updated: 20 May 2025

Summary

Definition

病史和体格检查

关键诊断因素

  • chronic erosive blistering of the skin, mucosa, or both
  • chronic mouth erosions (PV, PNP)
  • painful lips (PNP)
  • shortness of breath (PNP)
完整详情

其他诊断因素

  • pruritic scalp (PV, PF)
  • bloody nose (PV, PNP)
  • painful skin (PV, PF, PNP)
  • dysphagia (PV)
  • pruritic skin (PV, PF)
  • conjunctivitis (PV, PNP)
完整详情

危险因素

  • increasing age
  • HLA DR4 (PV)
  • HLA DQ1 (PV)
  • HLA DRB1 (PNP)
  • associated malignancy (PNP)
  • penicillamine use
  • ACE inhibitors use
  • penicillin use
  • tiopronin use
  • interleukin-2 use
  • nifedipine use
  • rifampin use
  • exposure to hematophagous insects
完整详情

诊断性检查

首要检查

  • skin biopsy, hematoxylin and eosin stain
  • skin biopsy, direct immunofluorescence
完整详情

需考虑的检查

  • indirect immunofluorescence on serum
  • serum ELISA
  • upper gastrointestinal endoscopy
  • CXR
  • chest CT scan
  • PFT
  • serum immunoblot (Western blot)
完整详情

治疗流程

急症处理

mild pemphigus vulgaris

mild pemphigus foliaceus

moderate to severe pemphigus vulgaris or pemphigus foliaceus

paraneoplastic pemphigus

持续性治疗

moderate to severe pemphigus vulgaris or pemphigus foliaceus 6 months after initial therapy: with disease control/complete remission

moderate to severe pemphigus vulgaris or pemphigus foliaceus 6 months after initial therapy: without complete remission

moderate to severe pemphigus vulgaris or pemphigus foliaceus 12 to 18 months after initial therapy: complete remission

severe/refractory pemphigus

撰稿人

作者

Jon H. Meyerle, MD

Associate Professor

Department of Dermatology

Uniformed Services University of the Health Sciences

Bethesda

MD

利益声明

JHM declares that he has no competing interests.

Grant J. Anhalt, MD

Professor

Department of Dermatology

Johns Hopkins Medical Institution

Baltimore

MD

利益声明

GJA declares that he has no competing interests.

同行评议者

Daniel Mimouni, MD

Professor

Department of Dermatology

Rabin Medical Center

Petah Tikva

Israel

Declarações

DM declares that he has no competing interests.

Vesna Petronic-Rosic, MD, MSc

Associate Professor and Clinical Director

Section of Dermatology

University of Chicago

Chicago

IL

Declarações

VPR declares that she has no competing interests.

Timothy Patton, MD

Assistant Professor of Dermatology

Department of Dermatology

University of Pittsburgh

Pittsburgh

PA

Declarações

TP declares that he has no competing interests.

Créditos aos pareceristas

Os tópicos do BMJ Best Practice são constantemente atualizados, seguindo os desenvolvimentos das evidências e das diretrizes. Os pareceristas aqui listados revisaram o conteúdo pelo menos uma vez durante a história do tópico.

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Referências

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Principais artigos

Joly P, Horvath B, Patsatsi Α, et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV). J Eur Acad Dermatol Venereol. 2020 Sep;34(9):1900-13.Texto completo  Resumo

Antiga E, Bech R, Maglie R, et al. S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol. 2023 Jun;37(6):1118-34.Texto completo  Resumo

Murrell DF, Peña S, Joly P, et al. Diagnosis and management of pemphigus: recommendations of an international panel of experts. J Am Acad Dermatol. 2020 Mar;82(3):575-85.e1.Texto completo  Resumo

Joly P, Maho-Vaillant M, Prost-Squarcioni C, et al. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet. 2017 May 20;389(10083):2031-40. Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.
  • Pemphigus images

  • Diagnósticos diferenciais

    • Familial benign pemphigus (Hailey-Hailey disease)
    • Bullous pemphigoid (BP)
    • Linear IgA bullous dermatosis
    Mais Diagnósticos diferenciais

  • Guidelines

    • Updated S2K guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)
    • S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)
    Mais Diretrizes
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