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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 aged 13-18 years, 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.

Codes

ICD10CM:
L10.0 – Pemphigus vulgaris

SNOMEDCT:
49420001 – Pemphigus vulgaris

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

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Best Tests

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Therapy

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Drug Reaction Data

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References

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Last Reviewed:01/09/2022
Last Updated:01/10/2022
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Pemphigus vulgaris in Child
See also in: Anogenital,Oral Mucosal Lesion
A medical illustration showing key findings of Pemphigus vulgaris : Crust, Face, Flaccid bullae, Nikolsky's sign, Oral erosions, Oral mucosa, Trunk, Skin erosions
Clinical image of Pemphigus vulgaris - imageId=759613. Click to open in gallery.  caption: 'Large erosions, healing with a purplish color (re-epithelialization) and surrounding brown postinflammatory macules on the back.'
Large erosions, healing with a purplish color (re-epithelialization) and surrounding brown postinflammatory macules on the back.
Copyright © 2024 VisualDx®. All rights reserved.