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

Pemphigus vulgaris in Adult

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 mucous membranes with or without skin involvement. It is typically characterized by the presence of circulating pathogenic autoantibodies (predominantly immunoglobulin [Ig]G4) against desmoglein, a cadherin-family, keratinocyte cell surface adhesion molecule of the desmosome, although other antibody subtypes and autoantibodies against other antigenic targets have been described. The target antigens in PV are desmoglein 3 (Dsg3) with or without desmoglein 1 (Dsg1). More than half of patients have both skin and mucosal involvement. In the mucosal-dominant type of PV, autoantibodies against Dsg3 (anti-Dsg3 Ig) are typically present, and in the mucocutaneous type, autoantibodies against Dsg1 and Dsg3 are typically present; however, variations in these patterns can be seen in the real-world setting.

The estimated incidence worldwide is 0.76-5 cases per million per year, although PV occurs in higher incidences in individuals of Jewish ancestry, as well as in certain geographic areas (Middle East, Southeastern Europe, and India). Variants of the ST18 gene have been found to confer increased risk of PV in some populations. PV is typically a disease of adults, with average onset between the ages of 40 and 60 years, but PV rarely can occur in childhood and young adulthood. There does not appear to be a consistent sex predilection.

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

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Pemphigus foliaceus
  • Drug-induced pemphigus (penicillamine, captopril, thiol-containing compounds)
  • IgG/IgA pemphigus – A rarely reported condition. It is unclear whether it represents a separate entity or pemphigus vulgaris that it transitioning to IgA pemphigus. Clinical findings in a recently reported series resembled pemphigus vulgaris, pemphigus foliaceus, and IgA pemphigus. In IgG/IgA pemphigus, dapsone can be used as an ancillary treatment.
  • Paraneoplastic pemphigus – Associated with underlying neoplasms (non-Hodgkin lymphoma, chronic lymphocytic leukemia, Castleman disease, thymomas, sarcomas, Waldenström macroglobulinemia).
  • Erythema multiforme – This condition may appear clinically identical to paraneoplastic pemphigus. Patients may have a history of previous episodes that resolved within 2-4 weeks. The presence of classic targetoid lesions of the skin is more consistent with erythema multiforme.
  • Bullous pemphigoid – Tense vesicles and bullae are the predominant feature of this condition, and only 20% of patients have oral involvement.
  • Stevens-Johnson syndrome – Usually drug induced and accompanied by high fever, skin tenderness, mucosal erosions, and desquamation 1-3 weeks after starting the inciting medication.
  • Reactive infectious mucocutaneous eruption (RIME) – Hemorrhagic crusting of lips is accompanied by a sparse macular, papular, or vesicular cutaneous eruption.
  • Staphylococcal and streptococcal Toxic shock syndrome and toxic shock-like syndrome – Look for sudden onset of exanthematous eruption.
  • Epidermolysis bullosa acquisita
  • Linear IgA bullous dermatosis
  • 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.
  • Oral ulcers of Systemic lupus erythematosus
  • Mucous membrane pemphigoid – This condition mostly affects the mucosa, most commonly the gingiva and palatal mucosa, with only 20% of patients showing cutaneous lesions.
  • Orofacial herpes simplex virus – The oral ulcerations may be diffuse but are composed of smaller lesions (1-2 mm) that coalesce.
  • Oral candidiasis
  • Aphthous stomatitis
  • Chemotherapy-induced mucositis
  • 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 bullous dermatosis – 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

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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:01/09/2022
Last Updated:02/16/2022
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Patient Information for Pemphigus vulgaris in Adult
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Pemphigus vulgaris in Adult
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.