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

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 (Dsg), a keratinocyte cell surface molecule, leading to dysfunctional cell adhesion between keratinocytes. The target antigens in PV are Dsg1 and Dsg3. 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 typically affects adults, with a mean age of onset in the 5th and 6th decades of life. Childhood PV, which refers to disease in children younger than 12 years of age, and juvenile PV, which refers to disease in adolescents 13-18 years of age, is rare, comprising less than 5% of all cases. Although there are 2 subtypes of PV, the mucosal-dominant type and mucocutaneous type, the mucocutaneous type is more frequently observed in children and adolescents.

PV is characterized by painful erosions on the oral mucosa and flaccid bullae and erosions 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 currently complications are related to the use of chronic 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 Child
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
Large erosions, healing with a purplish color (re-epithelialization) and surrounding brown postinflammatory macules on the back.
Copyright © 2019 VisualDx®. All rights reserved.