ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferencesInformation for PatientsView all Images (45)
Pemphigus vulgaris - Oral Mucosal Lesion
See also in: Overview,Anogenital
Print Captions OFF
Other Resources UpToDate PubMed

Pemphigus vulgaris - Oral Mucosal Lesion

See also in: Overview,Anogenital
Print Patient Handout Images (45)
Contributors: Christine S. Ahn MD, FAAD, William W. Huang MD, MPH, FAAD, Belinda Tan MD, PhD, Susan Burgin MD, Carl Allen DDS, MSD, Sook-Bin Woo MS, DMD, MMSc
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, a keratinocyte cell surface molecule, leading to disruption of normal cell adhesions between keratinocytes. The target antigens in PV are desmoglein (Dsg) 1 and 3. 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 affects men and women equally, and the mean age of onset is 50-60 years. The estimated incidence worldwide is 0.76-5 cases per million per year, although it occurs in higher incidences in individuals of Jewish ancestry. There are 2 subtypes of PV: the mucosal-dominant type and the mucocutaneous type. Almost all patients with PV develop painful erosions on the oral mucosa, and individuals with the mucocutaneous type develop flaccid bullae and erosions on the skin. Before the introduction of systemic corticosteroids, the mortality of PG was 75%. Still, severe cases of PV can be life threatening, and complications are related to the use of immunosuppression, secondary infection, loss of the skin barrier, and poor oral intake.

Patients usually complain of painful sores in the mouth and bleeding of the gums when brushing their teeth. Patients may complain of sore throat or pain with swallowing. The oral cavity is frequently the first site of involvement, with painful skin lesions sometimes appearing months or years later. Other mucosal sites of involvement include the nasal mucosa, throat, eyes, and genital areas.

For more information on familial pemphigus vulgaris, see OMIM.

Codes

ICD10CM:
L10.0 – Pemphigus vulgaris

SNOMEDCT:
49420001 – Pemphigus vulgaris

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Other entities that present with desquamative gingivitis that should be considered on the differential:
  • Lichen planus – Erosive oral lesions of lichen planus may resemble PV but can be distinguished by the presence of characteristic radiating reticulations or keratotic striae, known as Wickham's striae.
  • Oral ulcers of systemic lupus erythematosus
  • Mucous membrane pemphigoid – This condition mostly affects the mucosa and almost always the gingiva, although other sites may be involved. Only 20% of patients show cutaneous bullae.
  • Aphthous stomatitis – This would not involve the attached gingiva. Ulcers remit completely and then recur.
  • Primary herpes gingivostomatitis – The oral ulcerations may be diffuse but are comprised of smaller lesions (1-2 mm) that coalesce.
  • Plasma cell gingivitis – This hypersensitivity reaction presents as marked erythema of the gingiva (desquamative gingivitis) without blister formation.
  • Epidermolysis bullosa acquisita – This almost never presents intraorally without skin involvement.
  • Linear IgA disease – This almost never presents intraorally without skin involvement.
  • Chronic oral erythema multiforme – These ulcers remit completely and then recur and are associated with recurrent herpes simplex virus infection.
  • Bullous pemphigoid – This autoimmune bullous dermatosis is less likely to have oral involvement (20% of cases) and can be distinguished by histological and immunofluorescence studies.

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

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.

Subscription Required

References

Subscription Required

Last Reviewed: 04/09/2018
Last Updated: 04/09/2018
Copyright © 2019 VisualDx®. All rights reserved.
Pemphigus vulgaris - Oral Mucosal Lesion
See also in: Overview,Anogenital
Captions OFF Print 45 Images Filter Images
View all Images (45)
(with subscription)
 Reset
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.