Last reviewed: November 2017
Last updated: June  2017

Summary

Definition

History and exam

Key diagnostic factors

  • chronic erosive blistering of the skin, mucosa, or both
  • chronic mouth erosions (PV, PNP)
  • Nikolsky sign (PV, PF)
  • painful lips (PNP)
  • shortness of breath (PNP)

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)

Risk factors

  • HLA DR4 (PV)
  • HLA DQ1 (PV)
  • HLA DRB1 (PNP)
  • underlying malignancy (PNP)
  • D-penicillamine
  • captopril
  • enalapril
  • penicillin
  • thiopronine
  • interleukin 2
  • nifedipine
  • rifampicin
  • exposure to hematophagous insects

Diagnostic investigations

1st investigations to order

  • skin biopsy, hematoxylin and eosin stain
  • skin biopsy, direct immunofluorescence
Full details

Investigations to consider

  • indirect immunofluorescence on serum
  • serum ELISA
  • upper GI endoscopy
  • CXR
  • chest CT scan
  • PFT
  • serum immunoblot (Western blot)
  • serum immunoprecipitation
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Assistant Professor

Department of Dermatology

Uniformed Services University of the Health Sciences

Bethesda

MD

Disclosures

JHM declares that he has no competing interests.

Professor

Department of Dermatology

Johns Hopkins Medical Institution

Baltimore

MD

Disclosures

GJA declares that he has no competing interests.

Peer reviewers VIEW ALL

Professor

Department of Dermatology

Rabin Medical Center

Petah Tikva

Israel

Disclosures

DM declares that he has no competing interests.

Associate Professor and Clinical Director

Section of Dermatology

University of Chicago

Chicago

IL

Disclosures

VPR declares that she has no competing interests.

Assistant Professor of Dermatology

Department of Dermatology

University of Pittsburgh

Pittsburgh

PA

Disclosures

TP declares that he has no competing interests.

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