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

Pemphigus vulgaris - Anogenital in

See also in: Overview,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 skin and mucous membranes. It is typically characterized by the presence of circulating pathogenic autoantibodies (predominantly immunoglobulin G4 [IgG4]) 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 usually 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 of PV worldwide is 0.76-5 cases per million per year, although it occurs in higher incidences in individuals of Jewish ancestry as well as in certain geographic areas (Middle East, Southeastern Europe, and India). Variants of the ST18 gene have been found to confer increased risk of PV in some populations. 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. The mean age of onset for male anogenital pemphigus ranges from 32-67 years of age.

Almost all patients with PV develop painful erosions on the oral mucosa, and individuals with the mucocutaneous type develop flaccid bullae, erosions, and crusted erosions / plaques on the skin. Before the introduction of systemic corticosteroids, the mortality of PV was 75%. Still, 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.

Oral mucosal involvement is more common than genital involvement. The larynx, esophagus, conjunctiva, nasopharynx, and urethra can be involved rarely. The presence of genital lesions in men has only been described in a few case reports and small case series. It is less commonly observed than anogenital involvement in women. In rare cases, penile erosions can be the first manifestation of PV, followed by classic mucocutaneous findings. Lesions may be seen on the glans, shaft, and anus, as well as other mucosal sites.

Codes

ICD10CM:
L10.0 – Pemphigus vulgaris

SNOMEDCT:
49420001 – Pemphigus vulgaris

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

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:06/17/2020
Last Updated:02/16/2022
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Pemphigus vulgaris - Anogenital in
See also in: Overview,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 © 2022 VisualDx®. All rights reserved.