Summary
Definition
History and exam
Key diagnostic factors
- presence of risk factors
- chronic erosive blistering of the skin, mucosa, or both
- chronic mouth erosions (PV, PNP)
- 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
- 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
- rifampicin use
- exposure to haematophagous insects
Diagnostic investigations
1st investigations to order
- skin biopsy, haematoxylin and eosin stain
- skin biopsy, direct immunofluorescence
Investigations to consider
- indirect immunofluorescence on serum
- serum ELISA
- upper gastrointestinal endoscopy
- CXR
- chest CT scan
- PFT
- serum immunoblot (Western blot)
Treatment algorithm
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 declares that he has no competing interests.
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.
References
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 here.
Guidelines
- Consensus based Indian guidelines for the management of pemphigus vulgaris and pemphigus foliaceous
- S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)
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