Sections

Presentation

History

Mucous membranes

Pemphigus vulgaris presents with oral lesions in 50-70% of patients, and almost all patients have mucosal lesions at some point in the course of their disease. Mucosal lesions may be the sole sign for an average of 5 months before skin lesions develop, or they may be the sole manifestation of the disease. The diagnosis of pemphigus vulgaris should be considered in any patient with persistent oral erosive lesions.

Skin

Most patients with pemphigus vulgaris develop cutaneous lesions. The primary lesion of pemphigus vulgaris is a flaccid blister, which usually arises on healthy-appearing skin but may be found on erythematous skin. New blisters usually are flaccid or become flaccid quickly. Affected skin often is painful but rarely pruritic.

Drug-induced pemphigus vulgaris

Drugs reported most significantly in association with pemphigus vulgaris include penicillamine, captopril, cephalosporin, pyrazolones, nonsteroidal anti-inflammatory drugs (NSAIDs), and other thiol-containing compounds.^[23]Rifampin, emotional stress, thermal burns, ultraviolet rays, and infections (eg, coxsackievirus, Herpesviridae family) have also been reported as triggers for pemphigus vulgaris.^[24]

Physical Examination

Mucous membranes typically are affected first in pemphigus vulgaris. Mucosal lesions may precede cutaneous lesions by weeks or months. Patients with mucosal lesions may present to dentists, oral surgeons, or gynecologists.^{[25, 26]}

Mucous membranes

Intact bullae are rare in the mouth. More commonly, patients have ill-defined, irregularly shaped, gingival, buccal, or palatine erosions, which are painful and slow to heal. The erosions extend peripherally with shedding of the epithelium.

The mucous membranes most often affected in pemphigus vulgaris are those of the oral cavity, which is involved in almost all patients with pemphigus vulgaris and sometimes is the only area involved. Erosions may be seen on any part of the oral cavity. Erosions can be scattered and often are extensive. Erosions may spread to involve the larynx, with subsequent hoarseness. The patient often is unable to eat or drink adequately because the erosions are so uncomfortable.

In juvenile pemphigus vulgaris, stomatitis is the presenting complaint in more than 50% of the cases.

Other mucosal surfaces may be involved, including the conjunctiva,^[2]esophagus (causes odynophagia and/or dysphagia),^[3]labia, vagina, cervix, vulva,^[4]penis, urethra, nasal mucosa, and anus.

Skin

The primary lesion of pemphigus vulgaris is a flaccid blister filled with clear fluid that arises on healthy skin or on an erythematous base, as shown in the images below.

Early, small blister filled with clear fluid arises on healthy skin.

View Media Gallery

Flaccid blister filled with clear fluid arises on healthy skin.

View Media Gallery

The blisters are fragile; therefore, intact blisters may be sparse. The contents soon become turbid, or the blisters rupture, producing painful erosions, which is the most common skin presentation and is shown in the image below. Erosions often are large because of their tendency to extend peripherally with the shedding of the epithelium.

An erosion.

View Media Gallery

Vegetating pemphigus vulgaris

Ordinary pemphigus vulgaris erosions may develop vegetation. Lesions in skin folds readily form vegetating granulations. In some patients, erosions tend to develop excessive granulation tissue and crusting, and these patients display more vegetating lesions. This type of lesion tends to occur more frequently in intertriginous areas and on the scalp or face. The vegetating type of response can be more resistant to therapy and can remain in one place for long periods.

Nails

Acute or chronic paronychia, subungual hematomas, and nail dystrophies affecting one or several fingers or toes have been reported with pemphigus vulgaris.^{[5, 6]}Patients with paronychial pemphigus usually also have oral involvement.

Pemphigus in pregnancy

Pemphigus vulgaris occurring in pregnancy is rare. When present, maternal autoantibodies may cross the placenta, resulting in neonatal pemphigus. Neonatal pemphigus is transient and improves with clearance of maternal autoantibodies.^[27]Treatment of pemphigus vulgaris in pregnancy is with oral corticosteroids; however, prednisone and its metabolites cross the placenta and have been associated with low birth weight, prematurity, infection, and adrenal insufficiency.

Signs

For the Nikolsky sign in patients with active blistering, firm sliding pressure with a finger separates normal-appearing epidermis, producing an erosion. This sign is not specific for pemphigus vulgaris and is found in other active blistering diseases.

For the Asboe-Hansen sign, lateral pressure on the edge of a blister may spread the blister into clinically unaffected skin.

Causes

The cause of pemphigus vulgaris remains unknown; however, several potentially relevant factors have been identified.

Genetic factors

Predisposition to pemphigus is linked to genetic factors.^[28]Certain major histocompatibility complex (MHC) class II molecules, in particular alleles of human leukocyte antigen DR4 (DRB1*0402) and human leukocyte antigen DRw6 (DQB1*0503), are common in patients with pemphigus vulgaris.^{[29, 30, 31, 32, 33, 34]}

Age

Peak age of onset is from 50-60 years. Infants with neonatal pemphigus remit with clearance of maternal autoantibodies. The disease may develop in children or in older persons.

Disease association

Pemphigus occurs in patients with other autoimmune diseases, particularly myasthenia gravis and thymoma.^[35]A study of 110 patients with pemphigus found 4 patients with autoimmune thyroid disease and 3 patients with rheumatoid arthritis. In this study, however, autoimmune diseases were no more common in 969 first-degree relatives of patients with pemphigus than in the general population.^[36]

Differential Diagnoses

Ahmed AR, Moy R. Death in pemphigus. J Am Acad Dermatol. 1982 Aug. 7(2):221-8. [QxMD MEDLINE Link].
Hodak E, Kremer I, David M, et al. Conjunctival involvement in pemphigus vulgaris: a clinical, histopathological and immunofluorescence study. Br J Dermatol. 1990 Nov. 123(5):615-20. [QxMD MEDLINE Link].
Trattner A, Lurie R, Leiser A, et al. Esophageal involvement in pemphigus vulgaris: a clinical, histologic, and immunopathologic study. J Am Acad Dermatol. 1991 Feb. 24(2 Pt 1):223-6. [QxMD MEDLINE Link].
Marren P, Wojnarowska F, Venning V, Wilson C, Nayar M. Vulvar involvement in autoimmune bullous diseases. J Reprod Med. 1993 Feb. 38(2):101-7. [QxMD MEDLINE Link].
Berker DD, Dalziel K, Dawber RP, Wojnarowska F. Pemphigus associated with nail dystrophy. Br J Dermatol. 1993 Oct. 129(4):461-4. [QxMD MEDLINE Link].
Engineer L, Norton LA, Ahmed AR. Nail involvement in pemphigus vulgaris. J Am Acad Dermatol. 2000 Sep. 43(3):529-35. [QxMD MEDLINE Link].
Helander SD, Rogers RS 3rd. The sensitivity and specificity of direct immunofluorescence testing in disorders of mucous membranes. J Am Acad Dermatol. 1994 Jan. 30(1):65-75. [QxMD MEDLINE Link].
Diercks GF, Pas HH, Jonkman MF. Immunofluorescence of Autoimmune Bullous Diseases. Surg Pathol Clin. 2017 Jun. 10 (2):505-512. [QxMD MEDLINE Link].
Schmidt E, Spindler V, Eming R, et al. Meeting Report of the Pathogenesis of Pemphigus and Pemphigoid Meeting in Munich, September 2016. J Invest Dermatol. 2017 Jun. 137 (6):1199-1203. [QxMD MEDLINE Link].
Mentink LF, de Jong MC, Kloosterhuis GJ, Zuiderveen J, Jonkman MF, Pas HH. Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Br J Dermatol. 2007 Apr. 156(4):635-41. [QxMD MEDLINE Link].
Bhol K, Mohimen A, Ahmed AR. Correlation of subclasses of IgG with disease activity in pemphigus vulgaris. Dermatology. 1994. 189 Suppl 1:85-9. [QxMD MEDLINE Link].
Wilson CL, Wojnarowska F, Dean D, Pasricha JS. IgG subclasses in pemphigus in Indian and UK populations. Clin Exp Dermatol. 1993 May. 18(3):226-30. [QxMD MEDLINE Link].
Fitzpatrick RE, Newcomer VD. The correlation of disease activity and antibody titers in pemphigus. Arch Dermatol. 1980 Mar. 116(3):285-90. [QxMD MEDLINE Link].
Pietkiewicz P, Gornowicz-Porowska J, Bartkiewicz P, Bowszyc-Dmochowska M, Dmochowski M. Reviewing putative industrial triggering in pemphigus: cluster of pemphigus in the area near the wastewater treatment plant. Postepy Dermatol Alergol. 2017 Jun. 34 (3):185-191. [QxMD MEDLINE Link].
Pisanti S, Sharav Y, Kaufman E, Posner LN. Pemphigus vulgaris: incidence in Jews of different ethnic groups, according to age, sex, and initial lesion. Oral Surg Oral Med Oral Pathol. 1974 Sep. 38(3):382-7. [QxMD MEDLINE Link].
Simon DG, Krutchkoff D, Kaslow RA, Zarbo R. Pemphigus in Hartford County, Connecticut, from 1972 to 1977. Arch Dermatol. 1980 Sep. 116(9):1035-7. [QxMD MEDLINE Link].
Bastuji-Garin S, Souissi R, Blum L, et al. Comparative epidemiology of pemphigus in Tunisia and France: unusual incidence of pemphigus foliaceus in young Tunisian women. J Invest Dermatol. 1995 Feb. 104(2):302-5. [QxMD MEDLINE Link].
Hietanen J, Salo OP. Pemphigus: an epidemiological study of patients treated in Finnish hospitals between 1969 and 1978. Acta Derm Venereol. 1982. 62(6):491-6. [QxMD MEDLINE Link].
Wilson C, Wojnarowska F, Mehra NK, Pasricha JS. Pemphigus in Oxford, UK, and New Delhi, India: a comparative study of disease characteristics and HLA antigens. Dermatology. 1994. 189 Suppl 1:108-10. [QxMD MEDLINE Link].
Alexandru A, Zurac S, Salavastru CM, Andrei R, Tebeica T, Staniceanu F, et al. Direct immunofluorescence on hair follicles--present and future perspectives. Am J Dermatopathol. 2013 Jun. 35(4):472-6. [QxMD MEDLINE Link].
Rao R, Dasari K, Shenoi SD, Balachandran C, Dinesh P. Monitoring the disease activity in pemphigus by direct immunofluorescence of plucked hair: a pilot study. Indian J Dermatol. 2013 Mar. 58(2):164. [QxMD MEDLINE Link]. [Full Text].
Svecova D. Pemphigus vulgaris: a clinical study of 44 cases over a 20-year period. Int J Dermatol. 2015 Oct. 54 (10):1138-44. [QxMD MEDLINE Link].
Ayoub N. [Pemphigus and pemphigus-triggering drugs]. Ann Dermatol Venereol. 2005 Jun-Jul. 132(6-7 Pt 1):595. [QxMD MEDLINE Link].
Goldberg I, Ingher A, Brenner S. Pemphigus vulgaris triggered by rifampin and emotional stress. Skinmed. 2004 Sep-Oct. 3(5):294. [QxMD MEDLINE Link].
Ettlin DA. Pemphigus. Dent Clin North Am. 2005 Jan. 49(1):107-25, viii-ix. [QxMD MEDLINE Link].
Venugopal SS, Murrell DF. Diagnosis and clinical features of pemphigus vulgaris. Immunol Allergy Clin North Am. 2012 May. 32(2):233-43, v-vi. [QxMD MEDLINE Link].
Hern S, Vaughan Jones SA, et al. Pemphigus vulgaris in pregnancy with favourable foetal prognosis. Clin Exp Dermatol. 1998 Nov. 23(6):260-3. [QxMD MEDLINE Link].
Firooz A, Mazhar A, Ahmed AR. Prevalence of autoimmune diseases in the family members of patients with pemphigus vulgaris. J Am Acad Dermatol. 1994 Sep. 31(3 Pt 1):434-7. [QxMD MEDLINE Link].
Ahmed AR, Wagner R, Khatri K, et al. Major histocompatibility complex haplotypes and class II genes in non-Jewish patients with pemphigus vulgaris. Proc Natl Acad Sci U S A. 1991 Jun 1. 88(11):5056-60. [QxMD MEDLINE Link]. [Full Text].
Lombardi ML, Mercuro O, Ruocco V, et al. Common human leukocyte antigen alleles in pemphigus vulgaris and pemphigus foliaceus Italian patients. J Invest Dermatol. 1999 Jul. 113(1):107-10. [QxMD MEDLINE Link].
Reohr PB, Mangklabruks A, Janiga AM, DeGroot LJ, Benjasuratwong Y, Soltani K. Pemphigus vulgaris in siblings: HLA-DR4 and HLA-DQw3 and susceptibility to pemphigus. J Am Acad Dermatol. 1992 Aug. 27(2 Pt 1):189-93. [QxMD MEDLINE Link].
Szafer F, Brautbar C, Tzfoni E, et al. Detection of disease-specific restriction fragment length polymorphisms in pemphigus vulgaris linked to the DQw1 and DQw3 alleles of the HLA-D region. Proc Natl Acad Sci U S A. 1987 Sep. 84(18):6542-5. [QxMD MEDLINE Link]. [Full Text].
Matzner Y, Erlich HA, Brautbar C, et al. Identical HLA class II alleles predispose to drug-triggered and idiopathic pemphigus vulgaris. Acta Derm Venereol. 1995 Jan. 75(1):12-4. [QxMD MEDLINE Link].
Sinha AA, Brautbar C, Szafer F, et al. A newly characterized HLA DQ beta allele associated with pemphigus vulgaris. Science. 1988 Feb 26. 239(4843):1026-9. [QxMD MEDLINE Link].
Cruz PD Jr, Coldiron BM, Sontheimer RD. Concurrent features of cutaneous lupus erythematosus and pemphigus erythematosus following myasthenia gravis and thymoma. J Am Acad Dermatol. 1987 Feb. 16(2 Pt 2):472-80. [QxMD MEDLINE Link].
Leshem YA, Katzenelson V, Yosipovitch G, David M, Mimouni D. Autoimmune diseases in patients with pemphigus and their first-degree relatives. Int J Dermatol. 2011 Jul. 50(7):827-31. [QxMD MEDLINE Link].
Judd KP, Lever WF. Correlation of antibodies in skin and serum with disease severity in pemphigus. Arch Dermatol. 1979 Apr. 115(4):428-32. [QxMD MEDLINE Link].
Cholera M, Chainani-Wu N. Management of Pemphigus Vulgaris. Adv Ther. 2016 Jun. 33 (6):910-58. [QxMD MEDLINE Link].
Burmester IAK, Flaswinkel S, Thies CS, Kasprick A, Kamaguchi M, Bumiller-Bini V, et al. Identification of novel therapeutic targets for blocking acantholysis in pemphigus. Br J Pharmacol. 2020 Aug 19. [QxMD MEDLINE Link].
Zhang W, Wei S, Peng X, Xie S, Zhang Z, Zeng K, et al. Short-Term Intravenous Infusion of Cyclophosphamide in the Treatment of Refractory Pemphigus Vulgaris: A Retrospective Study. Dermatology. 2020 Aug 19. 1-6. [QxMD MEDLINE Link].
Lee MS, Yeh YC, Tu YK, Chan TC. Network meta-analysis-based comparison of first-line steroid-sparing adjuvants in treatment of pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol. 2020 Aug 13. [QxMD MEDLINE Link].
Chams-Davatchi C, Daneshpazhooh M. Prednisolone dosage in pemphigus vulgaris. J Am Acad Dermatol. 2005 Sep. 53(3):547. [QxMD MEDLINE Link].
Tabrizi MN, Chams-Davatchi C, Esmaeeli N, et al. Accelerating effects of epidermal growth factor on skin lesions of pemphigus vulgaris: a double-blind, randomized, controlled trial. J Eur Acad Dermatol Venereol. 2007 Jan. 21(1):79-84. [QxMD MEDLINE Link].
El Tal AK, Posner MR, Spigelman Z, Ahmed AR. Rituximab: a monoclonal antibody to CD20 used in the treatment of pemphigus vulgaris. J Am Acad Dermatol. 2006 Sep. 55(3):449-59. [QxMD MEDLINE Link].
Fatourechi MM, el-Azhary RA, Gibson LE. Rituximab: applications in dermatology. Int J Dermatol. 2006 Oct. 45(10):1143-55; quiz 1155. [QxMD MEDLINE Link].
Schmidt E, Hunzelmann N, Zillikens D, Brocker EB, Goebeler M. Rituximab in refractory autoimmune bullous diseases. Clin Exp Dermatol. 2006 Jul. 31(4):503-8. [QxMD MEDLINE Link].
Schmidt E, Seitz CS, Benoit S, Brocker EB, Goebeler M. Rituximab in autoimmune bullous diseases: mixed responses and adverse effects. Br J Dermatol. 2007 Feb. 156(2):352-6. [QxMD MEDLINE Link].
Leshem YA, David M, Hodak E, Waitman DA, Vardy D, Israeli M, et al. A prospective study on clinical response and cell-mediated immunity of pemphigus patients treated with rituximab. Arch Dermatol Res. 2013 Apr 17. [QxMD MEDLINE Link].
Balighi K, Daneshpazhooh M, Khezri S, Mahdavi-nia M, Hajiseyed-javadi M, Chams-Davatchi C. Adjuvant rituximab in the treatment of pemphigus vulgaris: a phase II clinical trial. Int J Dermatol. 2013 Jul. 52(7):862-7. [QxMD MEDLINE Link].
Boggs W. Fixed-Dose Rituximab Provides Durable Remission of Pemphigus. medscape [serial online]. Available at http://www.medscape.com/viewarticle/820533. Accessed: March 3, 2014.
Schmidt E. Rituximab as first-line treatment of pemphigus. Lancet. 2017 May 20. 389 (10083):1956-1958. [QxMD MEDLINE Link].
Joly P, Maho-Vaillant M, Prost-Squarcioni C, et al. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet. 2017 May 20. 389 (10083):2031-2040. [QxMD MEDLINE Link].
Lee J, Lundgren DK, Mao X, Manfredo-Vieira S, Nunez-Cruz S, Williams EF, et al. Antigen-specific B-cell depletion for precision therapy of mucosal pemphigus vulgaris. J Clin Invest. 2020 Aug 20. [QxMD MEDLINE Link].
el-Darouti M, Marzouk S, Abdel Hay R, et al. The use of sulfasalazine and pentoxifylline (low-cost antitumour necrosis factor drugs) as adjuvant therapy for the treatment of pemphigus vulgaris: a comparative study. Br J Dermatol. 2009 Aug. 161(2):313-9. [QxMD MEDLINE Link].
Boggs W. Methotrexate Effective as Adjuvant for Treating Pemphigus Vulgaris. Medscape. Jul 03 2013. Available at http://www.medscape.com/viewarticle/807342. Accessed: July 16, 2013.
Tran KD, Wolverton JE, Soter NA. Methotrexate in the treatment of pemphigus vulgaris: Experience in 23 patients. Br J Dermatol. 2013 Jun 18. [QxMD MEDLINE Link].
Werth VP, Fivenson D, Pandya AG, et al. Multicenter randomized, double-blind, placebo-controlled, clinical trial of dapsone as a glucocorticoid-sparing agent in maintenance-phase pemphigus vulgaris. Arch Dermatol. 2008 Jan. 144(1):25-32. [QxMD MEDLINE Link].
Quaresma MV, Bernardes Filho F, Hezel J, Peretti MC, Kac BK, Azulay-Abulafia L. Dapsone in the treatment of pemphigus vulgaris: adverse effects and its importance as a corticosteroid sparing agent. An Bras Dermatol. 2015 Jun. 90 (3 Suppl 1):51-4. [QxMD MEDLINE Link].
Yeh SW, Sami N, Ahmed RA. Treatment of pemphigus vulgaris: current and emerging options. Am J Clin Dermatol. 2005. 6(5):327-42. [QxMD MEDLINE Link].
Bystryn JC, Jiao D. IVIg selectively and rapidly decreases circulating pathogenic autoantibodies in pemphigus vulgaris. Autoimmunity. 2006 Nov. 39(7):601-7. [QxMD MEDLINE Link].
Green MG, Bystryn JC. Effect of intravenous immunoglobulin therapy on serum levels of IgG1 and IgG4 antidesmoglein 1 and antidesmoglein 3 antibodies in pemphigus vulgaris. Arch Dermatol. 2008 Dec. 144(12):1621-4. [QxMD MEDLINE Link].
Mittmann N, Chan B, Knowles S, Mydlarski PR, Cosentino L, Shear N. Effect of intravenous immunoglobulin on prednisone dose in patients with pemphigus vulgaris. J Cutan Med Surg. 2006 Sep-Oct. 10(5):222-7. [QxMD MEDLINE Link].
Mydlarski PR, Ho V, Shear NH. Canadian consensus statement on the use of intravenous immunoglobulin therapy in dermatology. J Cutan Med Surg. 2006 Sep-Oct. 10(5):205-21. [QxMD MEDLINE Link].
Amagai M, Ikeda S, Shimizu H, et al. A randomized double-blind trial of intravenous immunoglobulin for pemphigus. J Am Acad Dermatol. 2009 Apr. 60(4):595-603. [QxMD MEDLINE Link].
Asarch A, Razzaque Ahmed A. Treatment of juvenile pemphigus vulgaris with intravenous immunoglobulin therapy. Pediatr Dermatol. 2009 Mar-Apr. 26(2):197-202. [QxMD MEDLINE Link].
Bakos L, Zoratto G, Brunetto L, Mazzotti N, Cartell A. Photodynamic therapy: a useful adjunct therapy for recalcitrant ulceration in pemphigus vulgaris. J Eur Acad Dermatol Venereol. 2009 May. 23(5):599-600. [QxMD MEDLINE Link].
Oyama N, Togashi A, Nomura E, Kaneko F. Successful treatment with oral mizoribine in refractory ocular manifestation of mucosal pemphigus vulgaris: A unique response to different immunosuppressive drugs. Dermatol Ther. 2016 Jul 15. [QxMD MEDLINE Link].
Higashihara T, Kawase M, Kobayashi M, Hara M, Matsuzaki H, Uni R, et al. Evaluating the Efficacy of Double-Filtration Plasmapheresis in Treating Five Patients With Drug-Resistant Pemphigus. Ther Apher Dial. 2017 Jun. 21 (3):243-247. [QxMD MEDLINE Link].
Liu Y, Zhang B, Ma J, Wang H, Fan X, Zheng K, et al. Double-filtration plasmapheresis combined with immunosuppressive treatment for severe pemphigus: 10 years' experience of a single center in China. J Clin Apher. 2020 Aug 19. [QxMD MEDLINE Link].
Aberer W, Wolff-Schreiner EC, Stingl G, Wolff K. Azathioprine in the treatment of pemphigus vulgaris. A long-term follow-up. J Am Acad Dermatol. 1987 Mar. 16(3 Pt 1):527-33. [QxMD MEDLINE Link].
Baskan EB, Yilmaz M, Tunali S, Saricaoglu H. Efficacy and safety of long-term mycophenolate sodium therapy in pemphigus vulgaris. J Eur Acad Dermatol Venereol. 2009 Mar 17. [QxMD MEDLINE Link].
Jackson AP, Hall AG, McLelland J. Thiopurine methyltransferase levels should be measured before commencing patients on azathioprine. Br J Dermatol. 1997 Jan. 136(1):133-4. [QxMD MEDLINE Link].
Snow JL, Gibson LE. The role of genetic variation in thiopurine methyltransferase activity and the efficacy and/or side effects of azathioprine therapy in dermatologic patients. Arch Dermatol. 1995 Feb. 131(2):193-7. [QxMD MEDLINE Link].
Tavadia SM, Mydlarski PR, Reis MD, et al. Screening for azathioprine toxicity: a pharmacoeconomic analysis based on a target case. J Am Acad Dermatol. 2000 Apr. 42(4):628-32. [QxMD MEDLINE Link].
Beissert S, Mimouni D, Kanwar AJ, Solomons N, Kalia V, Anhalt GJ. Treating pemphigus vulgaris with prednisone and mycophenolate mofetil: a multicenter, randomized, placebo-controlled trial. J Invest Dermatol. 2010 Aug. 130(8):2041-8. [QxMD MEDLINE Link].
Strowd LC, Taylor SL, Jorizzo JL, Namazi MR. Therapeutic ladder for pemphigus vulgaris: emphasis on achieving complete remission. J Am Acad Dermatol. 2011 Mar. 64(3):490-4. [QxMD MEDLINE Link].
Ahmed AR, Spigelman Z, Cavacini LA, Posner MR. Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med. 2006 Oct 26. 355(17):1772-9. [QxMD MEDLINE Link].
Rituxan (rituximab) [package insert]. South San Francisco, Calif: Genentech, Inc. June 2018. Available at [Full Text].
Lolis M, Toosi S, Czernik A, Bystryn JC. Effect of intravenous immunoglobulin with or without cytotoxic drugs on pemphigus intercellular antibodies. J Am Acad Dermatol. 2011 Mar. 64(3):484-9. [QxMD MEDLINE Link].
Sharma VK, Khandpur S. Evaluation of cyclophosphamide pulse therapy as an adjuvant to oral corticosteroid in the management of pemphigus vulgaris. Clin Exp Dermatol. 2013 Aug. 38 (6):659-64. [QxMD MEDLINE Link].

Media Gallery

Early, small blister filled with clear fluid arises on healthy skin.
Flaccid blister filled with clear fluid arises on healthy skin.
An erosion.
Erosions and healing areas on the back.
Healing areas on the chest and abdomen.
Direct immunofluorescence showing intercellular immunoglobulin G throughout the epidermis of a patient with pemphigus vulgaris.

of 6

Author

Bassam Zeina, MD, PhD Consulting Staff, Department of Dermatology, Milton Keynes Hospital, UK

Bassam Zeina, MD, PhD is a member of the following medical societies: British Medical Association, Royal Society of Medicine, British Association of Dermatologists

Disclosure: Nothing to disclose.

Coauthor(s)

Nicole Sakka, MBBS Foundation Year 2, Royal Liverpool and Broadgreen University Hospital, Liverpool, UK

Disclosure: Nothing to disclose.

Sohail Mansoor, MBBS, MSc Dermatologist and Lead Physician in Dermatologic Surgery, Department of Dermatology, Barnet Hospital, UK

Sohail Mansoor, MBBS, MSc is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, Royal College of Physicians, American Academy of Anti-Aging Medicine, Royal College of Physicians and Surgeons of Glasgow

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Abby S Van Voorhees, MD Assistant Professor, Director of Psoriasis Services and Phototherapy Units, Department of Dermatology, University of Pennsylvania School of Medicine, Hospital of the University of Pennsylvania

Abby S Van Voorhees, MD is a member of the following medical societies: American Academy of Dermatology, Women's Dermatologic Society, National Psoriasis Foundation, American Medical Association, Phi Beta Kappa, Sigma Xi, The Scientific Research Honor Society

Disclosure: Received honoraria from Amgen for consulting; Received honoraria from Abbott for consulting; Partner received salary from Merck for management position; Received honoraria from Abbott for speaking and teaching; Received honoraria from Amgen for review panel membership; Received honoraria from Centocor for consulting; Received honoraria from Leo for consulting; Received none from Merck for other.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Mohsin Ali, MBBS, FRCP, MRCP, to the development and writing of this article.