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Pemphigus

  • მიმოხილვა
  • თეორია
  • დიაგნოზი
  • მართვა
  • მეთვალყურეობა
  • წყაროები
ბოლო მიმოხილვა: 17 Aug 2025
ბოლო განახლება: 20 May 2025

შეჯამება

定义

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

利益声明

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

Disclosures

VPR declares that she has no competing interests.

Timothy Patton, MD

Assistant Professor of Dermatology

Department of Dermatology

University of Pittsburgh

Pittsburgh

PA

Disclosures

TP declares that he has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

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.Full text  Abstract

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.Full text  Abstract

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.Full text  Abstract

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. Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
  • Pemphigus images

  • Differentials

    • Familial benign pemphigus (Hailey-Hailey disease)
    • Bullous pemphigoid (BP)
    • Linear IgA bullous dermatosis
    More Differentials

  • 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)
    More Guidelines
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