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General Medicine

Pemphigus Vulgaris

Tharani Ravi, MD, Elida M. Lopez Villa, MD and Andrea Padilla, MD Reviewed 06/2018
 


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—commonly the buccal and labial mucosa, palate, and tongue

  • Progression (weeks to months) to small, clear fluid–filled blisters on the s...

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

  • Minim...

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 OvidMedline]
2
Santoro FA, Stoop...

ADDITIONAL READING

Leshem YA, Gdalevich M, Ziv M, et al. Opportunistic infections in patients with pemphigus. J Am Acad Dermatol.  2014;71(2):284–292. 

CODES

ICD10

L10.0 Pemphigus vulgaris 

ICD9

694.4 Pemphigus 

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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Figure 14.78. Pemphigus vulgaris. A. Bulla. B. Erosion.
Figure 14.78. Pemphigus vulgaris. A. Bulla. B. Erosion.
Figure 14.78. Pemphigus vulgaris. A. Bulla. B. Erosion.
Figure 14.78. Pemphigus vulgaris. A. Bulla. B. Erosion.
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 un...
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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