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Pemphigus vulgaris in Adult
See also in: Anogenital,Oral Mucosal Lesion
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Pemphigus vulgaris in Adult

See also in: Anogenital,Oral Mucosal Lesion
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Contributors: Christine S. Ahn MD, FAAD, William W. Huang MD, MPH, FAAD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Pemphigus vulgaris (PV) is an acquired autoimmune bullous disease of the skin and mucous membranes. It is characterized by the presence of circulating IgG autoantibodies against desmoglein, a keratinocyte cell surface molecule, leading to disruption of normal cell adhesion between keratinocytes. The target antigens in PV are desmoglein (Dsg) 1 and 3. In the mucocutaneous type, autoantibodies against Dsg1 and Dsg3 are present, whereas the mucosal-dominant type of PV is characterized by autoantibodies against Dsg3.  

Pemphigus vulgaris affects men and women equally, and the mean age of onset is 50-60 years. The estimated incidence worldwide is 0.76-5 cases per million per year, although it occurs in higher incidences in individuals of Jewish ancestry. There are 2 subtypes of PV: the mucosal-dominant type and the mucocutaneous type. Almost all patients with PV develop painful erosions on the oral mucosa, and individuals with the mucocutaneous type develop flaccid bullae and erosions on the skin.

Before the introduction of systemic corticosteroids, the mortality of PG was 75%. Still, severe cases of PV can be life threatening, and complications are related to the use of immunosuppression, secondary infection, loss of the skin barrier, and poor oral intake.

For more information on familial pemphigus vulgaris, see OMIM.

Codes

ICD10CM:
L10.0 – Pemphigus vulgaris

SNOMEDCT:
49420001 – Pemphigus vulgaris

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Oral lesions:

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 04/09/2018
Last Updated: 04/20/2018
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Pemphigus vulgaris in Adult
See also in: Anogenital,Oral Mucosal Lesion
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Pemphigus vulgaris : Bullae, Crust, Flaccid bullae, Oral erosions, Widespread, Skin erosions
Clinical image of Pemphigus vulgaris
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