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

Pemphigus vulgaris in Child

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 skin and mucous membranes. It is characterized by the presence of circulating immunoglobulin G (IgG) autoantibodies against desmoglein (Dsg), a keratinocyte cell surface molecule, leading to dysfunction in 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.

PV typically affects adults, with a mean age of onset in the fifth and sixth decades of life. Variants of the ST18 gene have been found to confer increased risk of PV in some populations.

Childhood PV, which refers to disease in children younger than 12 years, and juvenile PV, which refers to disease in adolescents 13-18 years of age, are rare, comprising less than 5% of all cases. 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. 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:06/17/2020
Last Updated:06/20/2021
Copyright © 2021 VisualDx®. All rights reserved.
Pemphigus vulgaris in Child
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 © 2021 VisualDx®. All rights reserved.