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
Investigations to consider
- indirect immunofluorescence on serum
- serum ELISA
- upper GI endoscopy
- CXR
- chest CT scan
- PFT
- serum immunoblot (Western blot)
- serum immunoprecipitation
Treatment algorithm
Contributors
Authors
Associate 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
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.
Use of this content is subject to our disclaimer