Abstract
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.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Stanley JR, Amagai M. Pemphigus, bullous impetigo, and the staphylococcal scalded-skin syndrome. N Engl J Med. 2006;355:1800.
Mihai S, Sitaru C. Immunopathology and molecular diagnosis of autoimmune bullous diseases. J Cell Mol Med. 2007;11:462.
Kneisel A, Hertl M. Autoimmune bullous skin diseases. Part 1: clinical manifestations. J Dtsch Dermatol Ges. 2011;9:844.
Bystryn JC, Rudolph JL. Pemphigus. Lancet. 2005;366:61.
Venugopal SS, Murrell DF. Diagnosis and clinical features of pemphigus vulgaris. Dermatol Clin. 2011;29:373.
Sitaru C, Zillikens D. Mechanisms of blister induction by autoantibodies. Exp Dermatol. 2005;14:861.
Grando SA. Pemphigus autoimmunity: hypotheses and realities. Autoimmunity. 2012;45:7.
Amagai M, Tsunoda K, Zillikens D, et al. The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile. J Am Acad Dermatol. 1999;40:167.
Ding X, Aoki V, Mascaro JM Jr, et al. Mucosal and mucocutaneous (generalized) pemphigus vulgaris show distinct autoantibody profiles. J Invest Dermatol. 1997;109:592.
Firooz A, Mazhar A, Ahmed AR. Prevalence of autoimmune diseases in the family members of patients with pemphigus vulgaris. J Am Acad Dermatol. 1994;31:434.
Brenner S, Goldberg I. Drug-induced pemphigus. Clin Dermatol. 2011;29:455.
Ruocco V, Pisani M. Induced pemphigus. Arch Dermatol Res. 1982;274:123.
Ruocco V, Ruocco E, Lo Schiavo A, et al. Pemphigus: etiology, pathogenesis, and inducing or triggering factors: facts and controversies. Clin Dermatol. 2013;31:374.
Brenner S, Tur E, Shapiro J, et al. Pemphigus vulgaris: environmental factors. Occupational, behavioral, medical, and qualitative food frequency questionnaire. Int J Dermatol. 2001;40:562.
Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol. 2011;29:432.
James KA, Culton DA, Diaz LA. Diagnosis and clinical features of pemphigus foliaceus. Dermatol Clin. 2011;29:405.
Brenner S, Wohl Y. A survey of sex differences in 249 pemphigus patients and possible explanations. Skinmed. 2007;6:163.
Diaz LA, Sampaio SA, Rivitti EA, et al. Endemic pemphigus foliaceus (Fogo Selvagem): II. Current and historic epidemiologic studies. J Invest Dermatol. 1989;92:4.
Mustafa MB, Porter SR, Smoller BR, Sitaru C. Oral mucosal manifestations of autoimmune skin diseases. Autoimmun Rev. 2015;14:930.
Kavala M, TopaloÄŸlu Demir F, Zindanci I, et al. Genital involvement in pemphigus vulgaris (PV): correlation with clinical and cervicovaginal pap smear findings. J Am Acad Dermatol. 2015;73:655.
Kavala M, Altıntaş S, Kocatürk E, et al. Ear, nose and throat involvement in patients with pemphigus vulgaris: correlation with severity, phenotype and disease activity. J Eur Acad Dermatol Venereol. 2011;25:1324.
Torchia D, Romanelli P, Kerdel FA. Erythema multiforme and Stevens-Johnson syndrome/toxic epidermal necrolysis associated with lupus erythematosus. J Am Acad Dermatol. 2012;67:417.
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.
Shinkuma S, Nishie W, Shibaki A, et al. Cutaneous pemphigus vulgaris with skin features similar to the classic mucocutaneous type: a case report and review of the literature. Clin Exp Dermatol. 2008;33:724.
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.
Lebeau S, Müller R, Masouyé I, et al. Pemphigus herpetiformis: analysis of the autoantibody profile during the disease course with changes in the clinical phenotype. Clin Exp Dermatol. 2010;35:366.
Martin LK, Werth V, Villanueva E, et al. Interventions for pemphigus vulgaris and pemphigus foliaceus. Cochrane Database Syst Rev. 2009;(1):CD006263.
Beissert S, Werfel T, Frieling U, et al. A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of pemphigus. Arch Dermatol. 2006;142:1447.
Bystryn JC, Steinman NM. The adjuvant therapy of pemphigus. An update. Arch Dermatol. 1996;132:203.
Kasperkiewicz M, Schmidt E, Zillikens D. Current therapy of the pemphigus group. Clin Dermatol. 2012;30:84.
Atzmony L, Hodak E, Leshem YA, et al. The role of adjuvant therapy in pemphigus: a systematic review and meta-analysis. J Am Acad Dermatol. 2015;73:264.
Ratnam KV, Phay KL, Tan CK. Pemphigus therapy with oral prednisolone regimens. A 5-year study. Int J Dermatol. 1990;29:363.
Harman KE, Albert S, Black MM, British Association of Dermatologists. Guidelines for the management of pemphigus vulgaris. Br J Dermatol. 2003;149:926.
Murrell DF, Dick S, Ahmed AR, et al. Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus. J Am Acad Dermatol. 2008;58:1043.
Kneisel A, Hertl M. Autoimmune bullous skin diseases. Part 2: diagnosis and therapy. J Dtsch Dermatol Ges. 2011;9:927.
Hertl M. Pemphigus vulgaris. CME Dermatol. 2009;4:94.
Caldarola G, Kneisel A, Hertl M, Feliciani C. Herpes simplex virus infection in pemphigus vulgaris: clinical and immunological considerations. Eur J Dermatol. 2008;18:440.
Acknowledgments
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.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2018 Springer International Publishing AG, part of Springer Nature
About this chapter
Cite this chapter
Long, B., Koyfman, A. (2018). Pemphigus Vulgaris. In: Rose, E. (eds) Life-Threatening Rashes. Springer, Cham. https://doi.org/10.1007/978-3-319-75623-3_3
Download citation
DOI: https://doi.org/10.1007/978-3-319-75623-3_3
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-75622-6
Online ISBN: 978-3-319-75623-3
eBook Packages: MedicineMedicine (R0)