Pemphigus vulgaris is associated with high mortality if not treated. The classic clinical course is painful mucosal blisters and skin erosions. Mucosal lesions most commonly occur in the mouth, but any mucosal surface may be involved. Cutaneous blisters and bullae usually occur after mucosal lesions form, are typically painful and not pruritic, and are associated with positive Nikolsky sign. Fever is atypical and suggests superinfection. Diagnosis is clinical in the emergency department but can be confirmed with biopsy. Extensive lesions, severe superinfection, and toxic appearance require admission. Management includes systemic glucocorticoids, oral care, skin care, and adjunctive treatments as needed to facilitate resolution of lesions. Complications include electrolyte disorders, hypovolemia, secondary infection, and medication side effects. Superinfection can result in severe morbidity and mortality.
KeywordsPemphigus vulgaris Bullae Blister Nikolsky Rash Superinfection
The view(s) expressed herein are those of the author(s) and do not reflect the official policy or position of Brooke Army Medical Center, the US Army Medical Department, the US Army Office of the Surgeon General, the US Department of the Air Force, the US Department of the Army or the Department of Defense, or the US Government.
- 23.Amagai M. Pemphigus. In: Bolognia JL, Jorizzo JL, Schaffer JV, et al., editors. Dermatology, 3rd ed. vol 1. Elsevier, Saunders; 2012. p. 2776. ISBN: 9780702051821.Google Scholar
- 25.Payne AS, Stanley JR. Pemphigus. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K, editors. Fitzpatrick’s dermatology in general medicine, vol. 1. 8th ed. New York: McGraw Hill; 2012. https://accessmedicine.mhmedical.com/content.aspx?bookid=392§ionid=41138687. Accessed January 14, 2018.
- 27.Martin LK, Werth V, Villanueva E, et al. Interventions for pemphigus vulgaris and pemphigus foliaceus. Cochrane Database Syst Rev. 2009;(1):CD006263.Google Scholar
- 36.Hertl M. Pemphigus vulgaris. CME Dermatol. 2009;4:94.Google Scholar