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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


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.


L10.0 – Pemphigus vulgaris

49420001 – Pemphigus vulgaris

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Pemphigus foliaceus
  • Drug-induced pemphigus – Penicillamine, captopril, and thiol-containing compounds are common offending agents.
  • Paraneoplastic pemphigus – Associated with underlying neoplasms (Castleman disease is the most common in children and adolescents).
  • IgA pemphigus
  • Pemphigus herpetiformis
  • Erythema multiforme – Patients may have a history of previous episodes that resolved within 2-4 weeks. If classic targetoid lesions of the skin are present, this would be more consistent with erythema multiforme.
  • Reactive infectious mucocutaneous eruption (RIME) – Mucositis is a prominent feature and is usually associated with a sparse, polymorphic eruption.
  • Stevens-Johnson syndrome – Usually drug induced, accompanied by high fever, skin tenderness, mucosal erosions, and desquamation 1-3 weeks after the inciting medication is started.
  • Staphylococcal and streptococcal Toxic shock syndrome and toxic shock-like syndrome – Look for the sudden onset of an exanthematous eruption.
  • Epidermolysis bullosa acquisita
  • Linear IgA bullous dermatosis of childhood
  • Acute graft-versus-host disease – Clinical history of bone marrow transplantation.
  • Drug-induced hypersensitivity syndrome (drug reaction with eosinophilia and systemic symptoms [DRESS]) – Look for facial edema (hallmark of DRESS), eosinophilia, hepatitis, and other visceral involvement.
  • Subcorneal pustular dermatosis (Sneddon-Wilkinson syndrome)
  • Hailey-Hailey disease
  • Dermatitis herpetiformis
  • Erythroderma
  • Erythrodermic psoriasis
  • Atopic dermatitis with erythroderma
  • Allergic contact dermatitis
  • Sézary syndrome (see Cutaneous T-cell lymphoma)
  • Drug-induced phototoxic reaction
  • Drug-induced photoallergic reaction
Oral lesions:
  • Oral lichen planus – The erosive oral lesions of lichen planus are usually surrounded by characteristic radiating keratotic striae.
  • Mucous membrane pemphigoid – This condition mostly affects the mucosa at various sites, with only 20% of patients showing cutaneous involvement.
  • Orofacial herpes simplex virus (HSV) – The oral ulcerations may be diffuse but are composed of smaller lesions (1-2 mm) that coalesce.
  • Erythema multiforme – Hemorrhagic crusting of lips is typical. Intraoral erosions occur.
  • Reactive infectious mucocutaneous eruption – Mucositis resembling erythema multiforme is a prominent feature.
  • Oral candidiasis
  • Aphthous stomatitis
  • Cutaneous involvement in Orofacial herpes simplex virus would be unusual.

Best Tests

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Management Pearls

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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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Last Reviewed:01/09/2022
Last Updated:02/16/2022
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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.