Pemphigus

Last reviewed: 28 Aug 2022
Last updated: 13 Aug 2021

Summary

Definition

History and exam

Key diagnostic factors

  • chronic erosive blistering of the skin, mucosa, or both
  • chronic mouth erosions (PV, PNP)
  • painful lips (PNP)
  • shortness of breath (PNP)
More key diagnostic factors

Other diagnostic factors

  • pruritic scalp (PV, PF)
  • bloody nose (PV, PNP)
  • painful skin (PV, PF, PNP)
  • dysphagia (PV)
  • pruritic skin (PV, PF)
  • conjunctivitis (PV, PNP)
Other diagnostic factors

Risk factors

  • increasing age
  • HLA DR4 (PV)
  • HLA DQ1 (PV)
  • HLA DRB1 (PNP)
  • associated malignancy (PNP)
  • D-penicillamine
  • ACE inhibitors
  • penicillin
  • thiopronine
  • interleukin 2
  • nifedipine
  • rifampin
  • exposure to hematophagous insects
More risk factors

Diagnostic investigations

1st investigations to order

  • skin biopsy, hematoxylin and eosin stain
  • skin biopsy, direct immunofluorescence
More 1st investigations to order

Investigations to consider

  • indirect immunofluorescence on serum
  • serum ELISA
  • upper gastrointestinal endoscopy
  • CXR
  • chest CT scan
  • PFT
  • serum immunoblot (Western blot)
More investigations to consider

Treatment algorithm

ACUTE

mild pemphigus vulgaris

mild pemphigus foliaceus

moderate to severe pemphigus vulgaris or pemphigus foliaceus

paraneoplastic pemphigus

ONGOING

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

Contributors

Authors

Jon H. Meyerle, MD

Associate Professor

Department of Dermatology

Uniformed Services University of the Health Sciences

Bethesda

MD

Disclosures

JHM declares that he has no competing interests.

Grant J. Anhalt, MD

Professor

Department of Dermatology

Johns Hopkins Medical Institution

Baltimore

MD

Disclosures

GJA is a consultant for Janssen on a clinical trial design for an investigational drug for bullous pemphigoid.

Peer reviewers

Daniel Mimouni, MD

Professor

Department of Dermatology

Rabin Medical Center

Petah Tikva

Israel

Disclosures

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.

  • 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 pemphigus vulgaris and foliaceus initiated by the European Academy of Dermatology and Venereology (EADV)
    • Diagnosis and management of pemphigus: recommendations by an international panel of experts
    More Guidelines
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