Type your tag names separated by a space and hit enter

Pemphigus Vulgaris

Pemphigus Vulgaris is a topic covered in the 5-Minute Clinical Consult.

To view the entire topic, please or purchase a subscription.

Medicine Central™ is a quick-consult mobile and web resource that includes diagnosis, treatment, medications, and follow-up information on over 700 diseases and disorders, providing fast answers—anytime, anywhere. Explore these free sample topics:

Medicine Central

-- The first section of this topic is shown below --

Basics

Pemphigus is derived from the Greek word pemphix meaning “bubble” or “blister.” Pemphigus vulgaris (PV) is the most common form of pemphigus.

Description

  • Rare, potentially fatal autoimmune mucocutaneous blistering disease due to loss of keratinocyte to keratinocyte adhesion that involves the skin and the mucous membranes
  • Flaccid, painful, nonhealing bullae, pustules, or ulcerations that appear spontaneously on the skin and mucosal surfaces, typically begin in the oropharynx, and then may spread to the skin, having a predilection for the scalp, face, chest, axillae, groin, and pressure points
  • Patient often presents with erosions and no intact bullae.
  • System(s) affected: skin, GI, genitourinary

Epidemiology

Incidence
  • Disease of the middle-aged population, typically occurring after the age of 50 years, although some cases have been reported in younger adults and children
  • Affects both sexes equally
  • 0.1 to 3.2 cases per 100,000 individuals annually worldwide

Prevalence
  • Uncommon, affects <200,000 people in the United States
  • Seen more frequently in people of Mediterranean decent and Ashkenazi Jew

Etiology and Pathophysiology

  • Autoantibodies (IgG) are directed against desmoglein (Dsg) 1 and 3 adhesion molecules. Dsgs interact with desmosomes, which hold epidermal cells together. The antibodies against Dsg molecules cause intraepidermal blister formation and acantholysis.
  • Dsg 3 is predominantly expressed in oral epithelium, whereas both Dsg 1 and Dsg 3 are expressed in the skin.
  • Dsg 1 is expressed more intensely in the superficial layer, whereas Dsg 3 is found more abundantly in basal and suprabasal layers.
  • Additionally, autoantibodies against Dsg 4, the acetylcholine receptor, and pemphaxin have been identified in patients with PV. The exact pathogenesis of pemphigus has yet to be fully explained and is likely a “multiple hit” process. Autoimmune; stimulus is unknown.
  • Inducing factors include physical trauma, such as thermal burns, UV light, and ionizing radiation; neoplasm; emotional stress; drugs; and infections. Most patients lack a recognized inducing factor.

Genetics
Strong association with certain human leukocyte antigens (HLAs), especially HLA DR4, DR14, DRB1, DQB1, DQ1, and DQ3, although the susceptibility gene differs depending on ethnic origin. >95% of patients will test positive for HLA DR4 and/or HLA DR6 haplotypes.

Commonly Associated Conditions

  • Thymoma
  • Myasthenia gravis
  • Paraneoplastic pemphigus is a type of pemphigus defined by the fact that the patient has a malignancy at the time the pemphigus is diagnosed.
  • Gastric adenocarcinoma

-- To view the remaining sections of this topic, please or purchase a subscription --

Citation

Stephens, Mark B., et al., editors. "Pemphigus Vulgaris." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116441/all/Pemphigus_Vulgaris.
Pemphigus Vulgaris. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116441/all/Pemphigus_Vulgaris. Accessed April 18, 2019.
Pemphigus Vulgaris. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116441/all/Pemphigus_Vulgaris
Pemphigus Vulgaris [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 April 18]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116441/all/Pemphigus_Vulgaris.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Pemphigus Vulgaris ID - 116441 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116441/all/Pemphigus_Vulgaris PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -