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Pemphigus vulgaris in Adult
See also in: Anogenital,Oral Mucosal Lesion
Other Resources UpToDate PubMed

Pemphigus vulgaris in Adult

See also in: Anogenital,Oral Mucosal Lesion
Contributors: Erin X. Wei MD, 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 mucous membranes with or without skin involvement. It is typically characterized by the presence of circulating pathogenic autoantibodies (predominantly immunoglobulin [Ig]G4) against desmoglein, a cadherin-family, keratinocyte cell surface adhesion molecule of the desmosome, although other antibody subtypes and autoantibodies against other antigenic targets have been described. The target antigens in PV are desmoglein 3 (Dsg3) with or without desmoglein 1 (Dsg1). More than half of patients have both skin and mucosal involvement. In the mucosal-dominant type of PV, autoantibodies against Dsg3 (anti-Dsg3 Ig) are typically present, and in the mucocutaneous type, autoantibodies against Dsg1 and Dsg3 are typically present; however, variations in these patterns can be seen in the real-world setting.

The estimated incidence worldwide is 0.76-5 cases per million per year, although PV occurs in higher incidences in individuals of Jewish ancestry, as well as in certain geographic areas (Middle East, Southeastern Europe, and India). PV is typically a disease of adults, with average onset between the ages of 40 and 60 years, but PV rarely can occur in childhood and young adulthood. There does not appear to be a consistent gender predilection.

Severe cases of PV can be life-threatening, and complications can be related to immunosuppression from drugs used to treat severe PV, secondary infections, 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: 06/18/2020
Last Updated: 07/31/2020
Copyright © 2020 VisualDx®. All rights reserved.
Pemphigus vulgaris in Adult
See also in: Anogenital,Oral Mucosal Lesion
Pemphigus vulgaris : Crust, Face, Flaccid bullae, Nikolsky's sign, Oral erosions, Oral mucosa, Trunk, Skin erosions
Clinical image of Pemphigus vulgaris
Large erosions, healing with a purplish color (re-epithelialization) and surrounding brown postinflammatory macules on the back.
Copyright © 2020 VisualDx®. All rights reserved.