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General Medicine

Pemphigus Vulgaris

Tharani Ravi, MD and Jordan Kampschmidt, MD Reviewed 05/2023
 


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 mucoc...

DIAGNOSIS

HISTORY

  • Multiple, painful, nonhealing ulcers in the mouth (70%). Roughly 20% appears on genital mucosa. Rarely, erosions can appear in the conjunctivae.

  • Progression (weeks to months) to small,...

TREATMENT

Reduce inflammatory response and autoantibody production by suppression of the immune system to decrease blister formation and promote healing of blisters and erosions 

GENERAL MEASURES

  • Minimi...

ONGOING CARE

A minority of 10% achieve complete remission after initial treatment and do not need continued drug therapy; most require maintenance therapy to stay in remission. 

FOLLOW-UP RECOMMENDATIONS

REFERENCES

1
Joly  P, Litrowski  N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol.  2011;29(4):432–436. [View Abstract on Ovid...

CODES

ICD10

L10.0 Pemphigus vulgaris 

SNOMED

  • 49420001 Pemphigus vulgaris (disorder)

  • 403630005 Drug-induced pemphigus vulgaris (disorder)

  • 361212009 Oral pemphigus vulgaris (disorder)

CLINICAL PEARLS

PV is a rare, chronic, potentially fatal autoimmune vesiculobullous disease of the mucous membranes and skin. Biopsy immediately if suspected. When clinical suspicion is high, initiate...

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<bold><italic>Figure 14.78.</bold></bold> Pemphigus vulgaris. <bold>A.</bold> Bulla. <bold>B.</bold> Erosion.

 

<bold><italic>Figure 14.78.</bold></bold> Pemphigus vulgaris. <bold>A.</bold> Bulla. <bold>B....

<bold><italic>Figure 14.78.</bold></bold> Pemphigus vulgaris. <bold>A.</bold> Bulla. <bold>B.</bold> Erosion.

 

<bold><italic>Figure 14.78.</bold></bold> Pemphigus vulgaris. <bold>A.</bold> Bulla. <bold>B....

A pathogenetic mechanism of suprabasal dyshesion in pemphigus vulgaris. (1) A circulating autoantibody, whose stimulus for production is unknown, binds to an antigen on the outer leaflet of the plasma membranes (desmosomes) of keratinocytes, especially in the basal regions. (figure continues)

 

A pathogenetic mechanism of suprabasal dyshesion in pemphigus vulgaris. (1) A circulating autoantibody, whose stimulus for production is u...

Pemphigus vulgaris. Suprabasal dyshesion leads to an intraepidermal blister containing acantholytic keratinocytes.

 

Pemphigus vulgaris. Suprabasal dyshesion leads to an intraepidermal blister containing acantholytic keratinocytes.

 

FIGURE 52.5 Pemphigus vulgaris blisters on the forearm.

 

FIGURE 52.5 Pemphigus vulgaris blisters on the forearm.

 
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