Pemphigus Vulgaris

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Continuing Education Activity

Pemphigus vulgaris is a rare disease that causes blisters. It is the most common type of pemphigus. The mean age of onset is 50 to 60 years of age, and it affects both sexes equally. This activity reviews the role of the interprofessional team in the care of patients with this condition.


  • Identify the etiology of pemphigus vulgaris medical conditions and emergencies.
  • Outline the appropriate evaluation of pemphigus vulgaris.
  • Review the management options available for pemphigus vulgaris.
  • Describe interprofessional team strategies for improving care coordination and communication to advance pemphigus vulgaris and improve outcomes.


Pemphigus vulgaris (PV) is an autoimmune disease that results in blisters on cutaneous and mucosal surfaces.[1] Pemphigus is derived from pemphix, the Greek word for blister. Pemphigus was first described in 1788 by Stephen Dickson, who observed a patient with a blister on her tongue.[2] Although PV has not been shown to be contagious as initially thought, there have been possible triggers identified that might induce PV in patients with other autoimmune disorders.[1]


The etiology of pemphigus vulgaris is unknown, but patients are at risk that has a genetic predisposition. Several studies have linked PV with human leukocyte antigen (HLA) class II alleles. HLA-DRB1 0402 is associated with PV in Ashkenazi Jews, while DRB1 1401/04 and DQB1 0503 HLA alleles are associated with PV in non-Jewish patients of European or Asian descent.[3][4][5] Environmental factors in addition to diet, stress, viral infections, medications, radiation therapy, and allergens may all induce immune dysregulation leading to a flare of PV.[1] 


Though pemphigus vulgaris is prevalent worldwide, the occurrence of PV is related to ethnicity and geographic location. The reported incidence is between 0.1 and 0.5 per 100,000 people per year.[6] However, a higher rate is recorded in certain ethnicities.[7] Ashkenazi Jews have been found to have an increased incidence of PV, and the average onset of PV is usually seen between the ages of 40 to 60 years.[8] Also, within the demographics, people living in India, Southeast Europeans, and the Middle Eastern are at greatest risk for pemphigus Vulgaris.[9] The prevalence of PV is roughly the same in men and women. However, in Tunisia, PV is more common in women compared to men by a ratio of 4 to 1.[1][8]


Pemphigus vulgaris is caused by autoantibodies that target keratinocyte proteins (desmogleins). Acantholysis wherein there is a loss of keratinocyte to keratinocyte adhesion induced by the binding of circulating immunoglobulin G (IgG) autoantibodies to intercellular adhesion molecules.[10][11] Acantholysis is seen due to the autoantibodies destroying the intracellular connections leading to bullae that can easily rupture. A “super-compensation hypothesis” was recently submitted by Sinha et al. proposes that additional factors may also play a role in PV.[12] Multiple mechanisms for antibody-induced acantholysis have been suggested, including the induction of signal transduction and the inhibition of adhesive molecule function through steric hindrance, which can trigger cell separation.[13] Further, the pathogenesis was described in more detail by Hammers et al.[14] It has been found in patients with PV, the presence of autoantibodies against desmoglein 1 (Dsg 1) and desmoglein 3 (Dsg 3).[15] Desmogleins are transmembrane glycoproteins that are an integral part of desmosomes, which in part is required for cell-cell adhesion. Didona et al. reviewed how IgG binds desmogleins in greater detail. The most common targets on desmoglein for IgG antibodies are the extracellular cadherin domains, which can result in loss of desmosome adhesive properties, signaling pathways that trigger endocytosis and depletion, and direct inhibition of Dsg 3 trans-interactions.[16] 

Murine studies have shown that enzymatic inactivation of Dsg 1 and gene deletion of Dsg 3 results in pathology that is similar to PV. Also, the addition of IgG from patients with PV to mice results in pathology that is similar to PV. This phenomenon was observed to be dose-dependent and suggests that reducing the circulating levels of IgG against Dsg 1 and Dsg 3 can improve patient outcomes. In patients with the mucocutaneous disease, it was found that they have autoantibodies against Dsg 1 and Dsg 3, whereas patients with disease localized to their mucous membranes were only found to have autoantibodies against Dsg 3. This can be explained by the desmoglein compensation model in which there are Dsg 1 and Dsg 3 in the cutaneous epidermis and that having autoantibodies against just one of the desmogleins will not cause disease. However, due to the normal lack of Dsg 1 in mucous membranes, autoantibodies against only Dsg 3 will result in disease because Dsg 1 is not present to compensate for the inhibition of Dsg 3. The binding of antibodies to desmogleins has been confirmed by epitope mapping and is presumed to disrupt desmoglein binding by affecting steric hindrance. Another proposal of the pathophysiology in PV that may occur in addition to the above explanation is the desmoglein non-assembly depletion hypothesis. This theory suggests that autoantibodies not only bind desmoglein but that they also bind each other leading to crosslinking and the inability for desmosomes to maintain cell-cell adhesion. 

PV has been shown to have a genetic component, although familial cases are uncommon.[17] Patients with PV have been found to have a higher frequency of non-symptomatic first-degree relatives with circulating PV-IgG antibodies than compared to healthy controls. Additionally, first-degree relatives were found to have a higher prevalence of autoimmune diseases. DQB1*0503 and DRB1*0402 are two of the most common PV-associated alleles. DRB1*0402 was shown to be protective against rheumatoid arthritis.

Exposure to certain medications like penicillamine and captopril can trigger PV. Such a trigger can happen through the effects on binding to molecules involved in cell adhesion, influence on enzymes that mediate keratinocyte aggregation, and molecules involved in cell and by stimulating neoantigen formation.[18] Furthermore, nonsteroidal anti-inflammatory agents, penicillin, cephalosporins have also been associated with drug-induced PV.[18][19] Finally, controversial case reports associating PV with certain foods like red wine, garlic, leek, and peppers exists, though such association is not supported by robust evidence.[20]


The diagnosis of pemphigus vulgaris is confirmed with a biopsy of the lesion. Histopathology and Tzanck smear will reveal acantholysis. Direct immunofluorescence is considered the "gold standard" for the diagnosis of PV. Enzyme-linked immunosorbent assay (ELISA) testing will show serum IgG against Dsg 1, Dsg 3, or both in 98.5% of samples. ELISA testing for PV is commercially available.[8][21]

History and Physical

Pemphigus vulgaris is a blistering disease that initially presents on the oral mucosa in 80% of cases. These intraoral blisters often rupture, leaving painful erosions. Cutaneous lesions may appear in about 75% of patients with PV after the first oral blisters have presented. Vesicles, erosions, or bullae may appear on erythematous or normal-appearing skin. A Nikolsky sign is described as a blister formation with minor pressure or trauma and is seen in PV.[22] 

Due to the painful nature of oral lesions, PV can result in an impaired nutritional status. Mucosal PV can be found in the conjunctiva, nasal mucosa, larynx, pharynx, esophagus, penis, vagina, and anus.[23] Cutaneous lesions can be found most commonly on the face, trunk, groin, scalp, and armpits. PV usually spares palms and soles. Blisters can heal without scarring but may result in changes in the pigment. Alopecia may be observed when PV affects the scalp. Rarely, PV will involve nails when the disease is severe.[8][21] Rarely PV can present as pemphigus herpetiformis where PV can present with urticarial plaques and cutaneous vesicles arranged in a herpetiform.


The diagnosis of pemphigus vulgaris is ascertained by obtaining a thorough history and through the use of a biopsy. The biopsy for direct immunofluorescence (DIF) should be taken from normal-appearing perilesional skin or mucosa.[24] A physical exam will most likely be positive for mucosal involvement of oropharyngeal, nasal, and genital regions. Tzanck smear and histology examination will show acantholysis.[8] Serological studies like indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assay(ELISA) can detect circulating autoantibodies that bind epithelial cell surface antigens.[24]

In patients with positive DIF results, ELISA testing will show serum IgG against Dsg 1, Dsg 3, or both.[21] Additional workup should include vitals, pregnancy testing in select population, complete blood count (CBC), metabolic panel, antinuclear antibody (ANA), and urinalysis. A bone density scan may be needed early in the disease to ensure proper prevention of osteoporosis in at-risk patients. Also, quality of life and disease activity should be documented to ensure a baseline for further monitoring. Two validated severity scoring systems include the Pemphigus Disease Area Index (PDAI) and the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) that can be completed in 2 to 5 minutes.[25]

Treatment / Management

Systemic corticosteroids have had a significant impact on the treatment of pemphigus vulgaris and remain the backbone in the management of PV.[26] The first-line treatment of mild PV is systemic corticosteroids, which takes several weeks to achieve a response.[25] Tapering down of the dose can be initiated when symptoms are improved, but if reappearance of more than three lesions occurs, then dosing should be increased again to induce remission.[27] 

Second-line treatment is in combination with corticosteroids and includes adding either azathioprine or mycophenolate mofetil (MMF).[27] Azathioprine is a purine analog that inhibits purine synthesis. It can be administered orally or by intravenous infusion. Azathioprine should be discontinued if no improvements are seen within three months. Dosing should be adjusted based on renal function. Close monitoring is required to reduce the likelihood of side effects. Nausea is the most common side effect seen with azathioprine. Bone marrow suppression is also seen with azathioprine that can cause pancytopenia, thrombocytopenia, and leukopenia.[28] MMF functions as an immunosuppressant by inhibiting purine synthesis. It can be administered orally or by intravenous infusion. MMF is usually effective within two months of initiating treatment. Common side effects include nausea, vomiting, diarrhea, and abdominal discomfort. Intravenous administration can cause superficial thrombophlebitis and thrombosis.[27]

Anti-CD20 monoclonal antibodies, such as rituximab and ofatumumab, have also been used in conjunction with corticosteroids for first-line treatment in moderate-to-severe pemphigus.[21] Rituximab is an anti-CD20 monoclonal antibody that stops B lymphocytes from maturing into autoantibody-producing plasma cells. It is administered intravenously, and response is usually seen within three months. Common side effects include infusion-related reactions such as nausea, vomiting, headache, and fever.[27] A rare serious side effect of rituximab is progressive multifocal leukoencephalopathy (PML) that has also been seen in other monoclonal antibodies.[29]

Third-line treatments for PV include intravenous immunoglobulin (IVIG), cyclophosphamide, dapsone, immunoadsorption, and methotrexate.[27][30]

Most of the emerging therapies that could be effective for PV are monoclonal antibodies, with the cost being a major restriction to large trials. Obinutuzumab, ofatumumab, and veltuzumab are anti-CD20 monoclonal antibodies that may be an alternative to rituximab. Other therapies that may offer hope in PV include medications that target B-cell derived B-cell activating factor (BAFF), a proliferation-inducing ligand (APRIL), CD19, Bruton kinase (BTK), and interleukin (IL-4).[16]

Future PV research should focus on better understanding the specific pathogenic molecules and cytokines, after which better therapies can be produced.[31]

Differential Diagnosis

IgA pemphigus is similar to pemphigus vulgaris in that is has painful blisters, but IgA pemphigus does not present with oral mucosa blisters. Direct and indirect immunofluorescence can both help to differentiate PV from IgA pemphigus.[32]

Pemphigus foliaceus is similar to PV in the fact that it is an autoimmune blistering disease, however similarly to IgA pemphigus, pemphigus foliaceus does not affect the oral mucosa. Pemphigus foliaceus is less common than PV.[33]

Paraneoplastic pemphigus also presents with mucocutaneous vesicles and bullae similar to PV. Paraneoplastic pemphigus can be differentiated from PV using indirect immunofluorescence and immunoblot.[34]


Follow up, and response to treatment of pemphigus vulgaris should be monitored closely. Pemphigus is an active disease and often requires dose adjustments and change in medications according to the response to treatment. Septicemia is the leading cause of death in PV.[8] In a retrospective cohort study by Kridin et al. in Israel, it was found that survival rates were lower in patients with PV than compared with the general population. Patients who were diagnosed with PV at an older age had a lower survival rate. The median overall survival from the point of diagnosis was 10.1 years (0.2 to 29.8 years). There was not a statistically significant survival difference between men and women.[35]


Systemic corticosteroids, which are the backbone of pemphigus vulgaris treatment, are well known for causing osteoporosis and other complications. Corticosteroids have been reported to cause fractures in 30 to 50% and cause osteonecrosis in 9 to 40% of patients receiving long-term therapy.[36] The Fracture Risk Assessment (FRAX) is a tool used to further stratify the risk of osteoporotic fracture in patients that are not osteoporotic by T score and can be valuable when considering using pharmacologic treatment for the prevention of bone loss.[37] Other common side effects from corticosteroids include hyperglycemia, insomnia, increased appetite, hypertension, edema, adrenal suppression, cataracts, and delayed wound healing.[38]

A case-control study by Namazi et al. looked at the incidence of P-wave dispersion (PWD) in patients with PV. The authors reasoned that atrial fibrillation can be predicted by the presence of PWD and hypothesized that atrial fibrillation might be higher in PV patients. Patients were excluded if they had obesity, hyperlipidemia, hypertension, diabetes mellitus, and cardiopulmonary disease. The authors stated the limitations of their study included a small sample size of 90 patients, and the use of corticosteroids was higher in the patients with PV. Regardless of those limitations, the incidence of PWD confirmed by electrocardiogram (ECG) was higher in the PV group than in the control group.[39]

Deterrence and Patient Education

Patient education should include close follow-up visits with their provider and specialists. Medication compliance is integral to the management of pemphigus vulgaris and can be re-enforced by the support of the pharmacist.

Enhancing Healthcare Team Outcomes

Pemphigus vulgaris is a life-threatening autoimmune disease for which treatment is indicated. The goal of treatment in PV is to induce complete remission of PV with minimal treatment-related adverse effects. Cutaneous and mucosal involvement in PV can cause significant pain and functional impairment. Local measures may help to improve patient symptoms. Due to the serious adverse effects, prolonged treatment with high doses of a systemic glucocorticoid is not recommended.  A secondary infection like Herpes simplex should be considered when lesions fail to respond to treatment. An interprofessional approach, including social workers, wound care staff, dieticians, nursing, physician assistants, nurse practitioners, pharmacists, dentists, primary care physicians, hospitalists, and dermatologists, can optimize outcomes.

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Pemphigus Vulgaris
Pemphigus Vulgaris
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Article Details

Article Author

Curtis J. Ingold

Article Editor:

Moien AB Khan


2/11/2023 11:26:11 PM



Patel F, Wilken R, Patel FB, Sultani H, Bustos I, Duong C, Zone JJ, Raychaudhuri SP, Maverakis E. Pathophysiology of Autoimmune Bullous Diseases: Nature Versus Nurture. Indian journal of dermatology. 2017 May-Jun:62(3):262-267. doi: 10.4103/0019-5154.159620. Epub     [PubMed PMID: 28584368]


Dickson S. Observations on Pemphigus. The London medical journal. 1788:9(Pt 3):309-324     [PubMed PMID: 29139718]


Porro AM, Seque CA, Ferreira MCC, Enokihara MMSES. Pemphigus vulgaris. Anais brasileiros de dermatologia. 2019 Jul 29:94(3):264-278. doi: 10.1590/abd1806-4841.20199011. Epub 2019 Jul 29     [PubMed PMID: 31365654]


Firooz A, Mazhar A, Ahmed AR. Prevalence of autoimmune diseases in the family members of patients with pemphigus vulgaris. Journal of the American Academy of Dermatology. 1994 Sep:31(3 Pt 1):434-7     [PubMed PMID: 8077468]


Szafer F, Brautbar C, Tzfoni E, Frankel G, Sherman L, Cohen I, Hacham-Zadeh S, Aberer W, Tappeiner G, Holubar K. Detection of disease-specific restriction fragment length polymorphisms in pemphigus vulgaris linked to the DQw1 and DQw3 alleles of the HLA-D region. Proceedings of the National Academy of Sciences of the United States of America. 1987 Sep:84(18):6542-5     [PubMed PMID: 2888115]


Lombardi ML, Mercuro O, Ruocco V, Lo Schiavo A, Lombari V, Guerrera V, Pirozzi G, Manzo C. Common human leukocyte antigen alleles in pemphigus vulgaris and pemphigus foliaceus Italian patients. The Journal of investigative dermatology. 1999 Jul:113(1):107-10     [PubMed PMID: 10417627]


Langan SM, Smeeth L, Hubbard R, Fleming KM, Smith CJ, West J. Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study. BMJ (Clinical research ed.). 2008 Jul 9:337(7662):a180. doi: 10.1136/bmj.a180. Epub 2008 Jul 9     [PubMed PMID: 18614511]


Kneisel A, Hertl M. Autoimmune bullous skin diseases. Part 1: Clinical manifestations. Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG. 2011 Oct:9(10):844-56; quiz 857. doi: 10.1111/j.1610-0387.2011.07793.x. Epub     [PubMed PMID: 21955378]


Kridin K. Pemphigus group: overview, epidemiology, mortality, and comorbidities. Immunologic research. 2018 Apr:66(2):255-270. doi: 10.1007/s12026-018-8986-7. Epub     [PubMed PMID: 29479654]


Mihai S, Sitaru C. Immunopathology and molecular diagnosis of autoimmune bullous diseases. Journal of cellular and molecular medicine. 2007 May-Jun:11(3):462-81     [PubMed PMID: 17521373]


Hertl M, Eming R, Veldman C. T cell control in autoimmune bullous skin disorders. The Journal of clinical investigation. 2006 May:116(5):1159-66     [PubMed PMID: 16670756]


Sinha AA, Sajda T. The Evolving Story of Autoantibodies in Pemphigus Vulgaris: Development of the "Super Compensation Hypothesis". Frontiers in medicine. 2018:5():218. doi: 10.3389/fmed.2018.00218. Epub 2018 Aug 14     [PubMed PMID: 30155465]


Sitaru C, Zillikens D. Mechanisms of blister induction by autoantibodies. Experimental dermatology. 2005 Dec:14(12):861-75     [PubMed PMID: 16274453]


Hammers CM, Stanley JR. Mechanisms of Disease: Pemphigus and Bullous Pemphigoid. Annual review of pathology. 2016 May 23:11():175-97. doi: 10.1146/annurev-pathol-012615-044313. Epub 2016 Feb 22     [PubMed PMID: 26907530]


Amagai M, Tsunoda K, Zillikens D, Nagai T, Nishikawa T. The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile. Journal of the American Academy of Dermatology. 1999 Feb:40(2 Pt 1):167-70     [PubMed PMID: 10025740]


Didona D, Maglie R, Eming R, Hertl M. Pemphigus: Current and Future Therapeutic Strategies. Frontiers in immunology. 2019:10():1418. doi: 10.3389/fimmu.2019.01418. Epub 2019 Jun 25     [PubMed PMID: 31293582]


Vodo D, Sarig O, Sprecher E. The Genetics of Pemphigus Vulgaris. Frontiers in medicine. 2018:5():226. doi: 10.3389/fmed.2018.00226. Epub 2018 Aug 14     [PubMed PMID: 30155467]


Brenner S, Goldberg I. Drug-induced pemphigus. Clinics in dermatology. 2011 Jul-Aug:29(4):455-7. doi: 10.1016/j.clindermatol.2011.01.016. Epub     [PubMed PMID: 21679874]


Feng S, Zhou W, Zhang J, Jin P. Analysis of 6 cases of drug-induced pemphigus. European journal of dermatology : EJD. 2011 Sep-Oct:21(5):696-9. doi: 10.1684/ejd.2011.1428. Epub     [PubMed PMID: 21697060]


Ruocco V, Ruocco E, Lo Schiavo A, Brunetti G, Guerrera LP, Wolf R. Pemphigus: etiology, pathogenesis, and inducing or triggering factors: facts and controversies. Clinics in dermatology. 2013 Jul-Aug:31(4):374-381. doi: 10.1016/j.clindermatol.2013.01.004. Epub     [PubMed PMID: 23806154]


Schmidt E, Kasperkiewicz M, Joly P. Pemphigus. Lancet (London, England). 2019 Sep 7:394(10201):882-894. doi: 10.1016/S0140-6736(19)31778-7. Epub     [PubMed PMID: 31498102]


Silva SC, Nasser R, Payne AS, Stoopler ET. Pemphigus Vulgaris. The Journal of emergency medicine. 2019 Jan:56(1):102-104. doi: 10.1016/j.jemermed.2018.10.028. Epub 2018 Nov 30     [PubMed PMID: 30503724]


Pohla-Gubo G, Hintner H. Direct and indirect immunofluorescence for the diagnosis of bullous autoimmune diseases. Dermatologic clinics. 2011 Jul:29(3):365-72, vii. doi: 10.1016/j.det.2011.03.001. Epub     [PubMed PMID: 21605801]


Harman KE, Brown D, Exton LS, Groves RW, Hampton PJ, Mohd Mustapa MF, Setterfield JF, Yesudian PD. British Association of Dermatologists' guidelines for the management of pemphigus vulgaris 2017. The British journal of dermatology. 2017 Nov:177(5):1170-1201. doi: 10.1111/bjd.15930. Epub     [PubMed PMID: 29192996]


Hertl M, Jedlickova H, Karpati S, Marinovic B, Uzun S, Yayli S, Mimouni D, Borradori L, Feliciani C, Ioannides D, Joly P, Kowalewski C, Zambruno G, Zillikens D, Jonkman MF. Pemphigus. S2 Guideline for diagnosis and treatment--guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). Journal of the European Academy of Dermatology and Venereology : JEADV. 2015 Mar:29(3):405-14. doi: 10.1111/jdv.12772. Epub 2014 Oct 22     [PubMed PMID: 25338479]


Kridin K. Emerging treatment options for the management of pemphigus vulgaris. Therapeutics and clinical risk management. 2018:14():757-778. doi: 10.2147/TCRM.S142471. Epub 2018 Apr 27     [PubMed PMID: 29740210]


Mohammadi O, Kassim TA. Azathioprine. StatPearls. 2023 Jan:():     [PubMed PMID: 31194347]


Benjamin O, Goyal A, Lappin SL. Disease Modifying Anti-Rheumatic Drugs (DMARD). StatPearls. 2023 Jan:():     [PubMed PMID: 29939640]


Murrell DF, Peña S, Joly P, Marinovic B, Hashimoto T, Diaz LA, Sinha AA, Payne AS, Daneshpazhooh M, Eming R, Jonkman MF, Mimouni D, Borradori L, Kim SC, Yamagami J, Lehman JS, Saleh MA, Culton DA, Czernik A, Zone JJ, Fivenson D, Ujiie H, Wozniak K, Akman-Karakaş A, Bernard P, Korman NJ, Caux F, Drenovska K, Prost-Squarcioni C, Vassileva S, Feldman RJ, Cardones AR, Bauer J, Ioannides D, Jedlickova H, Palisson F, Patsatsi A, Uzun S, Yayli S, Zillikens D, Amagai M, Hertl M, Schmidt E, Aoki V, Grando SA, Shimizu H, Baum S, Cianchini G, Feliciani C, Iranzo P, Mascaró JM Jr, Kowalewski C, Hall R, Groves R, Harman KE, Marinkovich MP, Maverakis E, Werth VP. Diagnosis and management of pemphigus: Recommendations of an international panel of experts. Journal of the American Academy of Dermatology. 2020 Mar:82(3):575-585.e1. doi: 10.1016/j.jaad.2018.02.021. Epub 2018 Feb 10     [PubMed PMID: 29438767]


Bilgic A,Murrell DF, What is novel in the clinical management of pemphigus. Expert review of clinical pharmacology. 2019 Oct     [PubMed PMID: 31550941]


Aslanova M, Yarrarapu SNS, Zito PM. IgA Pemphigus. StatPearls. 2023 Jan:():     [PubMed PMID: 30085605]


Lepe K, Yarrarapu SNS, Zito PM. Pemphigus Foliaceus. StatPearls. 2023 Jan:():     [PubMed PMID: 29763004]


Kappius RH, Ufkes NA, Thiers BH. Paraneoplastic Pemphigus. StatPearls. 2023 Jan:():     [PubMed PMID: 31536300]


Kridin K,Zelber-Sagi S,Bergman R, Pemphigus Vulgaris and Pemphigus Foliaceus: Differences in Epidemiology and Mortality. Acta dermato-venereologica. 2017 Oct 2;     [PubMed PMID: 28536732]


Weinstein RS. Glucocorticoid-induced osteoporosis and osteonecrosis. Endocrinology and metabolism clinics of North America. 2012 Sep:41(3):595-611. doi: 10.1016/j.ecl.2012.04.004. Epub 2012 May 23     [PubMed PMID: 22877431]


Kavala M, Topaloğlu Demir F, Zindanci I, Can B, Turkoğlu Z, Zemheri E, Cam OH, Teksen A. Genital involvement in pemphigus vulgaris (PV): correlation with clinical and cervicovaginal Pap smear findings. Journal of the American Academy of Dermatology. 2015 Oct:73(4):655-9. doi: 10.1016/j.jaad.2015.06.057. Epub 2015 Jul 17     [PubMed PMID: 26194705]


Lane NE. Glucocorticoid-Induced Osteoporosis: New Insights into the Pathophysiology and Treatments. Current osteoporosis reports. 2019 Feb:17(1):1-7. doi: 10.1007/s11914-019-00498-x. Epub     [PubMed PMID: 30685820]


Puckett Y, Gabbar A, Bokhari AA. Prednisone. StatPearls. 2023 Jan:():     [PubMed PMID: 30521230]


Namazi N, Ariaeenejad S, Azad ME, Pishgahi M. Risk of Atrial Fibrillation in Pemphigus Vulgaris. Indian dermatology online journal. 2018 Jul-Aug:9(4):275-277. doi: 10.4103/idoj.IDOJ_205_17. Epub     [PubMed PMID: 30050823]


Drenovska K, Ivanova M, Vassileva S, Shahid MA, Naumova E. Association of specific HLA alleles and haplotypes with pemphigus vulgaris in the Bulgarian population. Frontiers in immunology. 2022:13():901386. doi: 10.3389/fimmu.2022.901386. Epub 2022 Aug 2     [PubMed PMID: 35983062]


Salviano-Silva A, Farias TDJ, Bumiller-Bini V, Castro MS, Lobo-Alves SC, Busch H, Pföhler C, Worm M, Goebeler M, van Beek N, Franke A, Wittig M, Zillikens D, de Almeida RC, Hundt JE, Boldt ABW, Ibrahim S, Augusto DG, Petzl-Erler ML, Schmidt E, Malheiros D. Genetic variability of immune-related lncRNAs: polymorphisms in LINC-PINT and LY86-AS1 are associated with pemphigus foliaceus susceptibility. Experimental dermatology. 2021 Jun:30(6):831-840. doi: 10.1111/exd.14275. Epub 2021 Jan 15     [PubMed PMID: 33394553]


Baker J, Seiffert-Sinha K, Sinha AA. Patient genetics shape the autoimmune response in the blistering skin disease pemphigus vulgaris. Frontiers in immunology. 2022:13():1064073. doi: 10.3389/fimmu.2022.1064073. Epub 2023 Jan 10     [PubMed PMID: 36703961]


Tavakolpour S. Pemphigus trigger factors: special focus on pemphigus vulgaris and pemphigus foliaceus. Archives of dermatological research. 2018 Mar:310(2):95-106. doi: 10.1007/s00403-017-1790-8. Epub 2017 Nov 6     [PubMed PMID: 29110080]


Crocker C. Radiation-induced pemphigus in a patient with an invasive ductal carcinoma of the breast: a case report. Oxford medical case reports. 2020 Jan:2020(1):omaa001. doi: 10.1093/omcr/omaa001. Epub 2020 Jan 31     [PubMed PMID: 32038873]


Jain VR, Mahajan RS, Rathi SS, Biyani VV, Ninama KR, Marfatia YS. Oral Mucosal Lesions - A Study of 369 Cases. Indian dermatology online journal. 2023 Mar-Apr:14(2):213-220. doi: 10.4103/idoj.idoj_203_22. Epub 2023 Mar 3     [PubMed PMID: 37089853]


Zhu Y, Su J, Zhang P, Deng M, Wu R, Liu Y, Su Y, Li S. The dysregulation of circulating innate lymphoid cells is related to autoantibodies in pemphigus vulgaris. International immunopharmacology. 2023 Apr:117():109921. doi: 10.1016/j.intimp.2023.109921. Epub 2023 Feb 24     [PubMed PMID: 36841156]


Celere BS, Vernal S, Brochado MJF, Segura-Muñoz SI, Roselino AM. Geographical foci and epidemiological changes of pemphigus vulgaris in four decades in Southeastern Brazil. International journal of dermatology. 2017 Dec:56(12):1494-1496. doi: 10.1111/ijd.13714. Epub 2017 Jul 24     [PubMed PMID: 28737218]


Alshami ML, Aswad F, Abdullah B. Desmogleins 1, 3, and E-cadherin immunohistochemical expression within mucocutaneous pemphigus vulgaris. The Pan African medical journal. 2022:42():186. doi: 10.11604/pamj.2022.42.186.35429. Epub 2022 Jul 7     [PubMed PMID: 36212929]


Ishii N. Significance of anti-desmocollin autoantibodies in pemphigus. The Journal of dermatology. 2023 Feb:50(2):132-139. doi: 10.1111/1346-8138.16660. Epub 2022 Dec 28     [PubMed PMID: 36578135]


Hartmann V, Hariton WV, Rahimi S, Hammers CM, Ludwig RJ, Müller EJ, Hundt JE. The human skin organ culture model as an optimal complementary tool for murine pemphigus models. Laboratory animals. 2023 Jan 16:():236772221145647. doi: 10.1177/00236772221145647. Epub 2023 Jan 16     [PubMed PMID: 36647613]


Boch K, Dräger S, Zillikens D, Hudemann C, Hammers CM, Patzelt S, Schmidt E, Langan EA, Eming R, Ludwig RJ, Bieber K. Immunization with desmoglein 3 induces non-pathogenic autoantibodies in mice. PloS one. 2021:16(11):e0259586. doi: 10.1371/journal.pone.0259586. Epub 2021 Nov 3     [PubMed PMID: 34731225]


Egu DT, Schmitt T, Waschke J. Mechanisms Causing Acantholysis in Pemphigus-Lessons from Human Skin. Frontiers in immunology. 2022:13():884067. doi: 10.3389/fimmu.2022.884067. Epub 2022 May 20     [PubMed PMID: 35720332]


Kawamura T, Muramatsu K, Orita A, Mai Y, Sugai T, Haga N, Fujimura Y, Miyauchi T, Izumi K, Koga H, Ishii N, Ujiie H. Two cases of Hallopeau-type pemphigus vegetans with anti-desmoglein 1 and anti-desmocollin 3 antibodies without mucosal involvement. Journal of the European Academy of Dermatology and Venereology : JEADV. 2023 Apr:37(4):e508-e510. doi: 10.1111/jdv.18704. Epub 2022 Nov 7     [PubMed PMID: 36305887]


Jindal A, Rao C, Pai SB, Rao R. Utility of oral mucosa as a substrate for the serodiagnosis of pemphigus: A descriptive analysis. Indian journal of dermatology, venereology and leprology. 2022 Mar-Apr:88(2):156-161. doi: 10.25259/IJDVL_469_20. Epub     [PubMed PMID: 34491669]


Ishii K, Yoshida K, Stanley JR, Yamagami J, Amagai M, Ishiko A. Pemphigus Vulgaris and Foliaceus IgG Autoantibodies Directly Block Heterophilic Transinteraction between Desmoglein and Desmocollin. The Journal of investigative dermatology. 2020 Oct:140(10):1919-1926.e7. doi: 10.1016/j.jid.2020.02.010. Epub 2020 Mar 3     [PubMed PMID: 32142800]


Schmitt T, Waschke J. Autoantibody-Specific Signalling in Pemphigus. Frontiers in medicine. 2021:8():701809. doi: 10.3389/fmed.2021.701809. Epub 2021 Aug 9     [PubMed PMID: 34434944]


Oktarina DA, van der Wier G, Diercks GF, Jonkman MF, Pas HH. IgG-induced clustering of desmogleins 1 and 3 in skin of patients with pemphigus fits with the desmoglein nonassembly depletion hypothesis. The British journal of dermatology. 2011 Sep:165(3):552-62. doi: 10.1111/j.1365-2133.2011.10463.x. Epub     [PubMed PMID: 21692763]


Amagai M, Karpati S, Prussick R, Klaus-Kovtun V, Stanley JR. Autoantibodies against the amino-terminal cadherin-like binding domain of pemphigus vulgaris antigen are pathogenic. The Journal of clinical investigation. 1992 Sep:90(3):919-26     [PubMed PMID: 1522242]


Sielski L, Baker J, DePasquale MC, Attwood K, Seiffert-Sinha K, Sinha AA. Desmoglein compensation hypothesis fidelity assessment in Pemphigus. Frontiers in immunology. 2022:13():969278. doi: 10.3389/fimmu.2022.969278. Epub 2022 Sep 23     [PubMed PMID: 36211362]


Garg R, Bhojani K. Non infective bullous lesions: a diagnostic challenge in a minimally equipped centre- based solely on microscopic findings. African health sciences. 2020 Jun:20(2):885-890. doi: 10.4314/ahs.v20i2.42. Epub     [PubMed PMID: 33163055]


Papara C, Danescu S, Rogojan L, Leucuta DC, Candrea E, Zillikens D, Baican A. Lymphocyte-predominant lesional inflammatory infiltrates of the skin are associated with mucosal-dominant phenotype in pemphigus. Journal of cutaneous pathology. 2023 Jan 21:():. doi: 10.1111/cup.14395. Epub 2023 Jan 21     [PubMed PMID: 36680509]


Gottesman SP, Abedi SM, Rosen JR, Gottlieb GJ. Hailey-Hailey-Like Pattern of Acantholysis on the Scalp Should Raise the Possibility of Incipient Pemphigus Vulgaris. The American Journal of dermatopathology. 2019 Apr:41(4):286-288. doi: 10.1097/DAD.0000000000001298. Epub     [PubMed PMID: 30640761]


See SHC, Peternel S, Adams D, North JP. Distinguishing histopathologic features of acantholytic dermatoses and the pattern of acantholytic hypergranulosis. Journal of cutaneous pathology. 2019 Jan:46(1):6-15. doi: 10.1111/cup.13356. Epub 2018 Nov 5     [PubMed PMID: 30203619]


Ng JKM, Cheung CM, Choi PCL, Ip EC, Li JJX, Chan AWS. A rare presentation of pemphigus vegetans as an isolated vegetative lesion-Review of histopathological clues and treatment effects in multiple biopsy specimens. Journal of cutaneous pathology. 2023 Mar:50(3):201-208. doi: 10.1111/cup.14375. Epub 2023 Jan 4     [PubMed PMID: 36502456]


Jabri H, Hali F, Elkaroini D, Mahdaoui S, Alatawna H, Chiheb S. Pemphigus vegetans-Neumann variant of the vulva. International journal of dermatology. 2023 Apr 26:():. doi: 10.1111/ijd.16702. Epub 2023 Apr 26     [PubMed PMID: 37098705]


Davarmanesh M, Zahed M, Sookhakian A, Jehbez S. Oral Pemphigus Vulgaris Treatment with Corticosteroids and Azathioprine: A Long-Term Study in Shiraz, Iran. Evidence-based complementary and alternative medicine : eCAM. 2022:2022():7583691. doi: 10.1155/2022/7583691. Epub 2022 Sep 17     [PubMed PMID: 36164397]


Korbl JD, Brusch A, Lucas M, von Nida J, Wood BA, Leecy TN, Harvey NT. A case of severe cutaneous and mucosal erosions. Clinical and experimental dermatology. 2020 Aug:45(6):780-782. doi: 10.1111/ced.14233. Epub 2020 May 14     [PubMed PMID: 32410330]


Yang M, Wu H, Zhao M, Long H, Lu Q. Vitamin D status in patients with autoimmune bullous dermatoses: a meta-analysis. The Journal of dermatological treatment. 2022 May:33(3):1356-1367. doi: 10.1080/09546634.2020.1810606. Epub 2020 Aug 26     [PubMed PMID: 32799714]


Lamichhane R, Chaudhary S. Delayed Diagnosis of Pemphigus Vulgaris Initially Presenting as an Oral Ulcer: A Case Report. JNMA; journal of the Nepal Medical Association. 2022 Jul 1:60(251):641-643. doi: 10.31729/jnma.7594. Epub 2022 Jul 1     [PubMed PMID: 36705190]


Hertl M, Veldman C. Pemphigus--paradigm of autoantibody-mediated autoimmunity. Skin pharmacology and applied skin physiology. 2001 Nov-Dec:14(6):408-18     [PubMed PMID: 11598441]


Xie D, Bilgic A, Abu Alrub N, Dicle Ö, Murrell DF. Clinical manifestations of alopecia in autoimmune blistering diseases: A cross-sectional study. JAAD international. 2023 Mar:10():6-13. doi: 10.1016/j.jdin.2022.08.025. Epub 2022 Oct 11     [PubMed PMID: 36387063]


Munhoz de Paula Alves Coelho K, Stall J, Henrique Condeixa de França P, Cristina de Carvalho Tavares L, Stefanello Bublitz G, Loos B, Carvalho Costa L, Fronza Júnior H. Pemphigus vulgaris of the cervix: diagnostic difficulties associated with the Pap test. Diagnostic cytopathology. 2015 Aug:43(8):635-7. doi: 10.1002/dc.23269. Epub 2015 Feb 26     [PubMed PMID: 25728997]


Akhyani M, Chams-Davatchi C, Naraghi Z, Daneshpazhooh M, Toosi S, Asgari M, Malekhami F. Cervicovaginal involvement in pemphigus vulgaris: a clinical study of 77 cases. The British journal of dermatology. 2008 Mar:158(3):478-82     [PubMed PMID: 18070212]


Helm M, Helm LA, Clebak KT, Foulke G. Autoimmune Skin Conditions: Autoimmune Blistering Disease. FP essentials. 2023 Mar:526():13-17     [PubMed PMID: 36913658]


Asokan S, Rai R, Boppe A, Umamaheswari G. Evaluation of the Role of Oral Mucosal Direct Immunofluorescence and Salivary Desmoglein 1 and 3 Enzyme-Linked Immunosorbent Assay in Patients With Oral Mucosal Pemphigus. Indian dermatology online journal. 2022 Sep-Oct:13(5):617-619. doi: 10.4103/idoj.idoj_44_22. Epub 2022 Sep 5     [PubMed PMID: 36304655]


Davis A, Dickson AL, Daniel LL, Nepal P, Zanussi J, Miller-Fleming TW, Straub PS, Wei WQ, Liu G, Cox NJ, Hung AM, Feng Q, Stein CM, Chung CP. Association Between Genetically Predicted Expression of TPMT and Azathioprine Adverse Events. Research square. 2023 Jan 13:():. pii: doi: 10.21203/ Epub 2023 Jan 13     [PubMed PMID: 36711487]


Sigmund AM, Winkler M, Engelmayer S, Kugelmann D, Egu DT, Steinert LS, Fuchs M, Hiermaier M, Radeva MY, Bayerbach FC, Butz E, Kotschi S, Hudemann C, Hertl M, Yeruva S, Schmidt E, Yazdi AS, Ghoreschi K, Vielmuth F, Waschke J. Apremilast prevents blistering in human epidermis and stabilizes keratinocyte adhesion in pemphigus. Nature communications. 2023 Jan 9:14(1):116. doi: 10.1038/s41467-022-35741-0. Epub 2023 Jan 9     [PubMed PMID: 36624106]


Garcia N, Patel OU, Graham L. Novel pain management therapies for patients with pemphigus. International journal of dermatology. 2023 Apr:62(4):575-578. doi: 10.1111/ijd.16331. Epub 2022 Jul 4     [PubMed PMID: 35781697]


Patil S, Mustaq S, Hosmani J, Khan ZA, Yadalam PK, Ahmed ZH, Bhandi S, Awan KH. Advancement in therapeutic strategies for immune-mediated oral diseases. Disease-a-month : DM. 2023 Jan:69(1):101352. doi: 10.1016/j.disamonth.2022.101352. Epub 2022 Mar 24     [PubMed PMID: 35339251]


Czerninski R, Zadik Y, Kartin-Gabbay T, Zini A, Touger-Decker R. Dietary alterations in patients with oral vesiculoulcerative diseases. Oral surgery, oral medicine, oral pathology and oral radiology. 2014 Mar:117(3):319-23. doi: 10.1016/j.oooo.2013.08.006. Epub 2013 Oct 18     [PubMed PMID: 24144994]


Hübner S, Sarhan M, Schauer F. Use of complementary and alternative medicine in patients with autoimmune bullous dermatoses: a cohort study analysis of a rare disease group. Complementary medicine research. 2023 Jan 16:():. doi: 10.1159/000529142. Epub 2023 Jan 16     [PubMed PMID: 36646063]


Paracha MM, Sagheer F, Khan AQ. A clinic-epidemiological study of 148 patients of pemphigus at Lady Reading Hospital, Peshawar: a case series. JPMA. The Journal of the Pakistan Medical Association. 2023 Mar:73(3):659-662. doi: 10.47391/JPMA.6039. Epub     [PubMed PMID: 36932776]


Maruyama S, Yamazaki M, Abé T, Kato Y, Kano H, Sumita Y, Tomihara K, Tanuma JI. Liquid-based cytology for differentiating two cases of pemphigus vulgaris from oral squamous cell carcinoma. Diagnostic cytopathology. 2023 May:51(5):E170-E175. doi: 10.1002/dc.25117. Epub 2023 Feb 10     [PubMed PMID: 36762831]


Miyachi H, Konishi T, Hashimoto Y, Matsui H, Fushimi K, Inozume T, Yasunaga H. Clinical course and outcomes of pemphigus vulgaris and foliaceus: A retrospective study using a nationwide database in Japan. The Journal of dermatology. 2023 Feb:50(2):212-221. doi: 10.1111/1346-8138.16641. Epub 2022 Nov 24     [PubMed PMID: 36424909]


Pankakoski A, Kluger N, Sintonen H, Panelius J. Clinical manifestations and comorbidities of pemphigus: a retrospective case-control study in southern Finland. European journal of dermatology : EJD. 2022 Jul 1:32(4):480-486. doi: 10.1684/ejd.2022.4266. Epub     [PubMed PMID: 36301748]


Karakioulaki M, Murrell DF, Kyriakou A, Patsatsi A. Investigation of comorbid autoimmune diseases in women with autoimmune bullous diseases: An interplay of autoimmunity and practical implications. International journal of women's dermatology. 2022 Oct:8(3):e053. doi: 10.1097/JW9.0000000000000053. Epub 2022 Oct 7     [PubMed PMID: 36225612]


Kadota H, Miyashita K, Fukushima S, Oryoji C, Hanada M, Yoshida S, Fujita H, Tachibana Y. Successful Management of a Severe Sacral Pressure Injury Penetrating to the Retroperitoneum. Wounds : a compendium of clinical research and practice. 2021 Mar:33(3):E24-E27     [PubMed PMID: 33914698]


Solanki VK, Nair PMK. Lifestyle medicine approach in managing pemphigus vulgaris: A case report. Explore (New York, N.Y.). 2023 Jan 7:():. pii: S1550-8307(23)00004-6. doi: 10.1016/j.explore.2023.01.003. Epub 2023 Jan 7     [PubMed PMID: 36646613]


Guptill JT, Sleasman JW, Steeland S, Sips M, Gelinas D, de Haard H, Azar A, Winthrop KL. Effect of FcRn antagonism on protective antibodies and to vaccines in IgG-mediated autoimmune diseases pemphigus and generalised myasthenia gravis. Autoimmunity. 2022 Dec:55(8):620-631. doi: 10.1080/08916934.2022.2104261. Epub 2022 Aug 29     [PubMed PMID: 36036539]


Seo JW, Park J, Lee J, Kim MY, Choi HJ, Jeong HJ, Lee JW, Jung SY, Kim WK. A case of pemphigus vulgaris associated with ulcerative colitis. Intestinal research. 2018 Jan:16(1):147-150. doi: 10.5217/ir.2018.16.1.147. Epub 2018 Jan 18     [PubMed PMID: 29422810]


Hocke J, Krauth J, Krause C, Gerlach S, Warnemünde N, Affeldt K, van Beek N, Schmidt E, Voigt J. Computer-aided classification of indirect immunofluorescence patterns on esophagus and split skin for the detection of autoimmune dermatoses. Frontiers in immunology. 2023:14():1111172. doi: 10.3389/fimmu.2023.1111172. Epub 2023 Feb 28     [PubMed PMID: 36926325]