Pemphigus Foliaceus: The Superficial Form of Pemphigus

Pemphigus foliaceus is a rare autoimmune blistering disease of the skin. The immune system makes antibodies against one skin protein, desmoglein 1, and the top layers of the skin lose their grip on each other. The blisters form so high in the skin that they break almost at once, so most people never see an intact blister at all. What they see instead are scaly, crusted, raw patches, often on the scalp, face and upper body. Unlike its better-known relative, pemphigus vulgaris, pemphigus foliaceus leaves the inside of the mouth alone.

This page covers why the blisters are so shallow, how the disease looks, the endemic form in rural Brazil known as fogo selvagem (Portuguese for “wild fire”) and the insects researchers have linked to it, cases set off by medicines, how the diagnosis is confirmed, the treatments that have been studied with their trial and cohort figures, what the long-term outlook looks like in published cohorts, and what is still not known.

Table of Contents

  1. Pemphigus Foliaceus in Brief
  2. Desmoglein 1 and Why the Blisters Stay Superficial
  3. How Pemphigus Foliaceus Looks on the Skin
  4. Fogo Selvagem: The Endemic Brazilian Form
  5. Insects, Rivers and the Search for a Trigger
  6. Pemphigus Set Off by Medicines
  7. How the Diagnosis Is Confirmed
  8. Corticosteroids and the Older Immunosuppressants
  9. Rituximab in Pemphigus Foliaceus
  10. Newer Antibody-Lowering Drugs
  11. Course and Outlook
  12. What Is Not Known
  13. Key Research Papers
  14. Connections
  15. Featured Videos

Pemphigus Foliaceus in Brief

Pemphigus is a family of diseases in which immunoglobulin G (IgG) antibodies attack the “rivets” that hold skin cells together. A 2017 review in Nature Reviews Disease Primers names three major subtypes: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. All three cause acantholysis, the medical word for skin cells coming apart from one another, which leads to blisters and raw erosions.

Pemphigus foliaceus is the shallowest of the three. A 2023 systematic review of biologic treatments describes it simply: it affects only the skin, and the mucous membranes are not involved. That one difference changes almost everything about how the disease looks, how it is told apart from other conditions, and how serious it tends to be.

There are two broad forms. The sporadic form turns up anywhere in the world, without any obvious pattern. The endemic form, fogo selvagem, clusters in rural parts of Brazil and other South American countries and has been studied for decades as a rare natural experiment in how something in the environment might switch on an autoimmune disease. Both forms share the same clinical and immunological features, according to a 2015 review by the Cooperative Group on Fogo Selvagem Research.

Before modern immune-suppressing treatment, pemphigus as a group was almost always fatal, as the 2020 European guideline on pemphigus vulgaris and foliaceus notes. That history explains why the disease is still treated seriously even though pemphigus foliaceus itself is the milder member of the family.

Desmoglein 1 and Why the Blisters Stay Superficial

Desmogleins are proteins that sit in desmosomes, the tiny spot-welds that join one skin cell to the next. Two of them matter in pemphigus: desmoglein 1 and desmoglein 3. The 2017 Nature Reviews paper explains that where the blisters form can be worked out from two things together: which desmoglein the antibodies target, and where in the body each desmoglein is made.

In pemphigus foliaceus, the antibodies are aimed at desmoglein 1. A 2011 review of the disease by James, Culton and Diaz describes desmoglein 1 as mainly expressed in the granular layer, one of the uppermost living layers of the epidermis. When antibodies knock it out, the cells there separate and form what pathologists call subcorneal blisters — splits just beneath the dead, flattened outer layer of the skin (the stratum corneum).

Two consequences follow from that:

The endemic Brazilian research adds a further detail. The 2015 review reports that the disease-causing antibodies in fogo selvagem belong mainly to the immunoglobulin G4 subclass and bind the outer tip of desmoglein 1 (its first and second extracellular domains). Harmless antibodies of a different subclass, immunoglobulin G1, bind a different part of the same protein (the fifth extracellular domain) and are found in people in the preclinical stage and in healthy neighbours living in endemic areas. The authors describe this shift from one part of the protein to another as “intramolecular epitope spreading.”

How Pemphigus Foliaceus Looks on the Skin

A 2017 case report in Anais Brasileiros de Dermatologia sums up the usual picture: scaly and crusty skin erosions involving the seborrheic areas — the parts of the skin richest in oil glands, which include the scalp, face, chest and upper back. The same report notes that a person can eventually become erythrodermic, meaning red and scaling over most of the body surface.

The 2018 review of fogo selvagem by Hans-Filho and colleagues describes the sequence. Flaccid blisters rupture readily to leave erosive, crusted lesions. These sometimes resemble seborrheic dermatitis, actinic keratosis and chronic cutaneous lupus erythematosus, which is one reason the disease can go unrecognised for some time. The presentation ranges from a few localized patches to widespread disease.

Localized pemphigus foliaceus is a recognised variant. A 2023 report from the United States described five cases and reviewed 18 earlier reported cases in the English literature. Most of the literature cases sat on sun-exposed skin such as the nose, cheeks, scalp and other areas of the face. Two of the reported patients progressed to generalized disease without treatment.

Pemphigus foliaceus can also take unusual forms. The same 2017 Brazilian case report describes a pustular presentation, with pus-filled spots that were hard to tell apart from generalized pustular psoriasis, in a man with psoriasis whose disease appeared after systemic corticosteroids were withdrawn. Rare patterns like this are one reason the diagnosis rests on the laboratory tests described further down rather than on appearance alone.

Fogo Selvagem: The Endemic Brazilian Form

Fogo selvagem is the name for pemphigus foliaceus where it occurs in endemic clusters. The 2018 review states that Brazil has the world’s largest number of patients, mainly in the Central-West region, and that the disease has also been reported in other South American countries. It affects young people and adults who have been exposed to rural areas, and familial cases occur.

The classic epidemiology was laid out in 1989 by the Cooperative Group on Fogo Selvagem Research. Their paper describes the disease as endemic in the Brazilian states of Goiás, Mato Grosso do Sul, Paraná, São Paulo and Minas Gerais, and apparently spreading toward the northwest and west. The people at risk were young peasants or children of either sex and any race exposed to the local ecology in rural areas. Cases in cities were rare. The group noted that fogo selvagem commonly appears in wild areas being colonized and disappears as those areas become urbanized, and that a significant number of cases occurred in families with several genetically related members affected.

Genes matter too. The 2015 review reports that the human leukocyte antigen (HLA) alleles DRB1*0404, *1402, *1406 and *0102 had been identified as risk factors, with a relative risk above 14.

The picture is changing. A cross-sectional study from a university hospital in Minas Gerais, published in 2024, enrolled 122 people with pemphigus: 64 with endemic pemphigus foliaceus and 58 with pemphigus vulgaris. The two groups differed significantly in the age at which the disease began, occupation, area of residence, and in having forests and rivers or streams close to the home. Comparing their endemic patients with a study at the same service in 2008, the authors found a significant difference in area of residence. They conclude that, historically, endemic pemphigus foliaceus cases have fallen in this population while pemphigus vulgaris cases have risen. The authors note that their hospital is not a reference centre for the whole state.

Because fogo selvagem clusters in particular landscapes, researchers have spent decades looking for an environmental trigger. The research points mainly at blood-feeding insects, though none has been proven to cause the disease.

Black flies

The 1989 epidemiology paper reported that most patients lived close to rivers and within the 10–15 km flying range of mosquitoes or black flies (such as Simulium), and put forward the hypothesis that a black fly might be the vector that sets the disease off. A 1992 case-control study at the Hospital do Pênfigo in Goiânia compared 52 people with fogo selvagem against 52 people with other skin diseases, matched for age, sex and occupation. Of the risk factors examined — black fly bites, rodents at home, cereal dust, dust or fumes from clearing trees and shrubs, and insecticides — only black fly bites showed an odds ratio significantly different from one, at 4.7.

The Limão Verde reservation

A long-running study on the Terena reservation of Limão Verde, a community of approximately 1,200 people with a high rate of the disease, began surveillance in 1994 and had documented 43 cases by its 2004 report. The researchers found that many healthy residents, not only patients, carried antibodies against desmoglein 1. The homes of patients were rustic, with thatched roofs and walls and dirt floors, and had a high frequency of blood-feeding insects such as bedbugs and kissing bugs. The predominant black fly on the reservation was Simulium nigrimanum. The authors concluded that the environmental trigger may be linked to exposure to blood-feeding insects.

Sand fly saliva

A 2012 study added a specific molecular candidate. Clusters of fogo selvagem overlap with clusters of leishmaniasis, a disease spread by the sand fly Lutzomyia longipalpis. The researchers found that antibodies from people with fogo selvagem recognise sand fly salivary proteins, specifically one called LJM11, and that anti-desmoglein 1 antibodies taken from patients cross-react with LJM11. Mice immunized with LJM11 went on to make anti-desmoglein 1 antibodies. The authors proposed that insect bites may deliver salivary proteins that start a cross-reactive antibody response in genetically susceptible people.

A 2016 follow-up from the same group examined the immunoglobulin G4 antibodies cloned from patients and found that all of them cross-reacted with both desmoglein 1 and LJM11. The 2015 review frames the overall idea as “antigenic mimicry” — an immune response to something from outside the body that happens to resemble a body protein — and presents it as a possible explanation, not a settled one.

Pemphigus Set Off by Medicines

Some cases of pemphigus, including pemphigus foliaceus, follow the start of a medicine. A 2021 systematic review collected 170 people with drug-induced pemphigus from 134 published studies. Their mean age was 57.19 years, and the disease appeared within a mean of 154.27 days.

The review’s authors conclude that, despite looking the same as classic pemphigus under the microscope and on the skin, most drug-induced cases are less severe and have a better prognosis. The figures above combine all subtypes; the review does not give them separately for pemphigus foliaceus.

How the Diagnosis Is Confirmed

The 2017 Nature Reviews paper states that diagnosis is based on the clinical picture and confirmed with tissue and antibody tests. A 2024 review of autoimmune blistering diseases from Lübeck describes the modern toolkit.

Skin biopsy

Standard microscopy of a fresh lesion shows the split high in the epidermis, just under the outer layer, with cells that have come apart from one another. This subcorneal split lies higher in the skin than the split seen in pemphigus vulgaris.

Direct immunofluorescence

The 2024 review states that direct immunofluorescence microscopy of a biopsy taken from skin beside a lesion is still the diagnostic gold standard for autoimmune blistering diseases. A fluorescent dye linked to anti-antibodies shows immunoglobulin G deposited around the skin cells themselves.

Desmoglein antibody blood tests

Enzyme-linked immunosorbent assay (ELISA) tests measure antibodies against specific desmogleins in the blood. The 2024 review lists desmoglein 1 as the target antigen used for pemphigus foliaceus and desmoglein 3 for pemphigus vulgaris, and describes these assays as highly specific and sensitive. A typical pemphigus foliaceus result is antibodies against desmoglein 1 without antibodies against desmoglein 3.

The same test can follow the disease over time. A French study at 13 university hospitals followed 26 people with pemphigus for 17 months. Antibody levels against desmoglein 1 fell significantly after initial treatment and tracked the course of the skin lesions. A cutoff of 20 units per millilitre gave a 79% positive predictive value and an 84% negative predictive value for a skin relapse. Antibody levels against desmoglein 3 did not track the course of mouth lesions as well.

Corticosteroids and the Older Immunosuppressants

The 2017 Nature Reviews paper describes the standard first-line treatment for pemphigus at that time as systemic corticosteroids plus “adjuvant” therapies: immunosuppressive drugs, intravenous immunoglobulin and plasmapheresis (filtering antibodies out of the blood). The 2018 review of fogo selvagem says much the same for the endemic form: treatment generally involves systemic corticosteroids along with immunosuppressants to reduce corticosteroid side effects. Most published trials pooled pemphigus foliaceus with pemphigus vulgaris, and pemphigus foliaceus patients were usually the smaller group.

Topical treatment for limited disease

In the 2023 report of five localized cases, patients were treated with topical corticosteroids and calcineurin inhibitors (another class of anti-inflammatory creams), and some also received systemic corticosteroids and dapsone. Each responded; two reached remission, and none relapsed over a mean follow-up of 3.7 years. The authors suggest that localized pemphigus foliaceus may be controlled with topical therapy and systemic dapsone. This is a small case series, not a trial.

What the randomized trials show

A 2014 systematic review and meta-analysis pooled 20 randomized trials with 826 participants with pemphigus vulgaris or pemphigus foliaceus. Most were small and open-label. Its findings, drug by drug:

The authors concluded that none of these treatments raised the complete response rate compared with glucocorticoids alone, which were then the mainstay of treatment.

Methotrexate

A 2022 retrospective study from New York followed 34 people with pemphigus vulgaris or pemphigus foliaceus who took methotrexate for at least three months. The median time to tapering prednisone down to 5 mg was 161 days, and to stopping it completely 308 days. Relapses were common (88.2%). At last follow-up, 26.5% were managed on topical therapies alone. Adverse events led to stopping methotrexate in 2.9%.

Pulse therapy in a Brazilian cohort

A 2023 Brazilian cohort study looked at 37 people with pemphigus vulgaris and 41 with pemphigus foliaceus who had not responded to usual treatments and were given monthly high-dose dexamethasone pulses, with or without cyclophosphamide. The people with pemphigus foliaceus had had their disease longer before pulse therapy began and needed more monthly pulses to reach remission in the first phase (median 10 versus 6). After completed cycles, 34.1% of the pemphigus foliaceus group were in remission, 12.2% relapsed and 24.4% dropped out. Outcomes were similar between the two diseases.

Corticosteroid side effects

Long courses of corticosteroids carry their own harms. In the Ritux 3 trial described in the next section, the most common severe (grade 3–4) adverse events in both groups were diabetes and endocrine disorders, myopathy (muscle weakness) and bone disorders, and severe events were more frequent in the group given prednisone alone.

Rituximab in Pemphigus Foliaceus

Rituximab is a monoclonal antibody that removes B cells, the white blood cells that mature into antibody factories. The 2017 Nature Reviews paper called it a promising option that might soon become first-line therapy. The 2020 European guideline states that rituximab has since been approved as a first-line treatment for moderate and severe pemphigus vulgaris in Europe and the United States.

The Ritux 3 trial

The French Ritux 3 trial, published in 2017, randomly assigned 90 people with newly diagnosed pemphigus to prednisone alone, tapered over 12 or 18 months, or to rituximab plus a short course of prednisone. The trial gave 1,000 mg of rituximab into a vein on days 0 and 14, then 500 mg at months 12 and 18, with prednisone at 0.5 or 1.0 mg/kg per day tapered over 3 or 6 months. At month 24, 41 of 46 people (89%) in the rituximab group were in complete remission off all therapy, compared with 15 of 44 (34%) on prednisone alone. No patient died. Severe adverse events were more frequent with prednisone alone (53 events in 29 patients) than with rituximab plus prednisone (27 events in 16 patients). The US label for rituximab states that this trial enrolled 74 people with pemphigus vulgaris and 16 with pemphigus foliaceus.

What the US label covers

The US prescribing information for rituximab lists moderate to severe pemphigus vulgaris in adults as an approved use; pemphigus foliaceus is not listed. For pemphigus vulgaris, the label gives two 1,000 mg infusions two weeks apart with a tapering course of glucocorticoids, then 500 mg at month 12 and every 6 months after that or based on clinical evaluation. The label carries a boxed warning for fatal infusion-related reactions (approximately 80% of fatal reactions occurred with the first infusion), severe mucocutaneous reactions, hepatitis B virus reactivation, and progressive multifocal leukoencephalopathy, a rare brain infection.

Pooled data in pemphigus foliaceus

Because so few people with pemphigus foliaceus are enrolled in trials, the best foliaceus-specific evidence comes from pooled case series and cohorts.

An international expert panel, whose recommendations were published in 2020 after a Delphi survey of 54 experts from 21 countries, included intravenous CD20 inhibitors (the class that contains rituximab) as a first-line therapy option for moderate-to-severe pemphigus.

A German retrospective study of 59 people with pemphigus, followed for a median of 104 months, gives a sense of the longer course after rituximab. Complete remission off therapy was reached by 39% after the first cycle and 61% with further courses; 27% stayed in remission off therapy long term. Relapse after the first cycle occurred in 63%, and renewed rituximab brought back complete remission in 94% of relapses. In that study, pemphigus subtype did not differ between the people who reached remission off therapy and those who did not.

Newer Antibody-Lowering Drugs

Because pemphigus is driven by immunoglobulin G antibodies, drugs that lower antibody levels have been tested. Efgartigimod alfa blocks the neonatal Fc receptor, which normally recycles immunoglobulin G and keeps it in circulation.

The phase III ADDRESS trial, published in 2026, randomized 222 adults with moderate-to-severe pemphigus (190 with pemphigus vulgaris and 32 with pemphigus foliaceus) to weekly efgartigimod alfa with hyaluronidase injected under the skin or placebo, with everyone also taking prednisone starting at 0.5 mg/kg daily. The trial gave 2,000 mg on days 1 and 8, then 1,000 mg weekly. The drug quickly lowered total immunoglobulin G and the antibodies against desmoglein 1 and desmoglein 3, but this did not improve disease-activity scores. In the pemphigus vulgaris group, complete remission on minimal prednisone by week 30 was reached by 35.5% on the drug and 30.3% on placebo, a difference that was not statistically significant. Adverse events occurred in 89.1% on the drug and 76.0% on placebo, with serious events in 12.2% and 13.3%. The authors conclude the drug did not show clinical benefit over corticosteroids alone at 30 weeks. The trial’s primary measure was set in the pemphigus vulgaris group; the abstract gives no separate result for the 32 people with pemphigus foliaceus.

Course and Outlook

The 2018 review describes the course of fogo selvagem as usually chronic. Once the disease is under control, many patients stay stable on low-dose medication, and a significant proportion reach remission.

Mortality figures for pemphigus foliaceus are reassuring compared with pemphigus vulgaris, though the cohorts are small:

Relapse is a regular part of the disease. The rituximab meta-analysis above reported a pooled relapse rate of 38.0% in pemphigus foliaceus, and the French ELISA study showed that rising desmoglein 1 antibodies tended to come before a skin flare.

What Is Not Known

Pemphigus foliaceus is a diagnosis made with a biopsy and laboratory tests, and decisions about its treatment belong with a clinician, usually a dermatologist experienced with blistering diseases.

Key Research Papers

  1. Kasperkiewicz M, Ellebrecht CT, Takahashi H, Yamagami J, Zillikens D, Payne AS, et al. Pemphigus. Nat Rev Dis Primers. 2017;3:17026. PubMed PMID: 28492232
  2. James KA, Culton DA, Diaz LA. Diagnosis and clinical features of pemphigus foliaceus. Dermatol Clin. 2011;29(3):405-12, viii. PubMed PMID: 21605805
  3. Hans-Filho G, Aoki V, Bittner NRH, Bittner GC. Fogo selvagem: endemic pemphigus foliaceus. An Bras Dermatol. 2018;93(5):638-650. PubMed PMID: 30156612
  4. Aoki V, Rivitti EA, Diaz LA. Update on fogo selvagem, an endemic form of pemphigus foliaceus. J Dermatol. 2015;42(1):18-26. PubMed PMID: 25558948
  5. Diaz LA, Sampaio SA, Rivitti EA, Martins CR, Cunha PR, Lombardi C, et al. Endemic pemphigus foliaceus (Fogo Selvagem): II. Current and historic epidemiologic studies. J Invest Dermatol. 1989;92(1):4-12. PubMed PMID: 2642512
  6. Lombardi C, Borges PC, Chaul A, Sampaio SA, Rivitti EA, Friedman H, et al. Environmental risk factors in endemic pemphigus foliaceus (Fogo selvagem). "The Cooperative Group on Fogo Selvagem Research". J Invest Dermatol. 1992;98(6):847-50. PubMed PMID: 1593148
  7. Aoki V, Millikan RC, Rivitti EA, Hans-Filho G, Eaton DP, Warren SJ, et al. Environmental risk factors in endemic pemphigus foliaceus (fogo selvagem). J Investig Dermatol Symp Proc. 2004;9(1):34-40. PubMed PMID: 14870983
  8. Qian Y, Jeong JS, Maldonado M, Valenzuela JG, Gomes R, Teixeira C, et al. Cutting Edge: Brazilian pemphigus foliaceus anti-desmoglein 1 autoantibodies cross-react with sand fly salivary LJM11 antigen. J Immunol. 2012;189(4):1535-9. PubMed PMID: 22798673
  9. Qian Y, Jeong JS, Ye J, Dang B, Abdeladhim M, Aoki V, et al. Overlapping IgG4 Responses to Self- and Environmental Antigens in Endemic Pemphigus Foliaceus. J Immunol. 2016;196(5):2041-50. PubMed PMID: 26826247
  10. Barcelos VM, Vale ECSD, Araujo MG, Bittencourt FV. Epidemiological and clinical study of cases of endemic pemphigus foliaceus and pemphigus vulgaris in a reference center in the state of Minas Gerais, Brazil. An Bras Dermatol. 2024;99(1):43-52. PubMed PMID: 37805349
  11. Sousa VB, Santana CNLEL, Pereira DDN, Gripp AC. Pemphigus foliaceus with pustular presentation in a patient with psoriasis. An Bras Dermatol. 2017;92(5 Suppl 1):115-117. PubMed PMID: 29267466
  12. Kunadia A, Moschella S, McLemore J, Sami N. Localized Pemphigus Foliaceus: Diverse Presentations, Treatment, and Review of the Literature. Indian J Dermatol. 2023;68(1):123. PubMed PMID: 37151255
  13. Ghaedi F, Etesami I, Aryanian Z, Kalantari Y, Goodarzi A, Teymourpour A, et al. Drug-induced pemphigus: A systematic review of 170 patients. Int Immunopharmacol. 2021;92:107299. PubMed PMID: 33418246
  14. van Beek N, Holtsche MM, Atefi I, Olbrich H, Schmitz MJ, Pruessmann J, et al. State-of-the-art diagnosis of autoimmune blistering diseases. Front Immunol. 2024;15:1363032. PubMed PMID: 38903493
  15. Abasq C, Mouquet H, Gilbert D, Tron F, Grassi V, Musette P, et al. ELISA testing of anti-desmoglein 1 and 3 antibodies in the management of pemphigus. Arch Dermatol. 2009;145(5):529-35. PubMed PMID: 19451496
  16. Atzmony L, Hodak E, Gdalevich M, Rosenbaum O, Mimouni D. Treatment of pemphigus vulgaris and pemphigus foliaceus: a systematic review and meta-analysis. Am J Clin Dermatol. 2014;15(6):503-15. PubMed PMID: 25403548
  17. Kolla A, Shah P, Cymerman R, Fruchter R, Adotama P, Soter NA. Assessing the use of methotrexate as an alternate therapy for pemphigus vulgaris and pemphigus foliaceus. Dermatol Ther. 2022;35(8):e15661. PubMed PMID: 35734997
  18. Fontenelle LF, Bueno-Filho R, Vernal S, Delfino R, Barbosa GSL, Donadi EA, et al. Dexamethasone-cyclophosphamide pulse therapy outcomes comparing pemphigus vulgaris and pemphigus foliaceus groups in a Brazilian cohort study. An Bras Dermatol. 2023;98(6):774-780. PubMed PMID: 37355353
  19. Joly P, Maho-Vaillant M, Prost-Squarcioni C, Hebert V, Houivet E, Calbo S, 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;389(10083):2031-2040. PubMed PMID: 28342637
  20. Kieffer C, Hebert V, Joly P, Tedbirt B. Efficacy and Safety of Rituximab in Pemphigus Foliaceus: A Systematic Review and Meta-analysis. Dermatol Ther (Heidelb). 2026;16(8):3831-3845. PubMed PMID: 42390708
  21. Carver CA, Kalesinskas M, Ahmed AR. Current biologics in treatment of pemphigus foliaceus: a systematic review. Front Immunol. 2023;14:1267668. PubMed PMID: 37901249
  22. Carver C, Kalesinskas M, Dheden N, Ahmed AR. Treatment of Pediatric Pemphigus Foliaceus. Cureus. 2023;15(9):e45373. PubMed PMID: 37779684
  23. Shimanovich I, Baumann T, Schmidt E, Zillikens D, Hammers CM. Long-term outcomes of rituximab therapy in pemphigus. J Eur Acad Dermatol Venereol. 2020;34(12):2884-2889. PubMed PMID: 32367562
  24. Murrell DF, Peña S, Joly P, Marinovic B, Hashimoto T, Diaz LA, et al. Diagnosis and management of pemphigus: Recommendations of an international panel of experts. J Am Acad Dermatol. 2020;82(3):575-585.e1. PubMed PMID: 29438767
  25. Joly P, Horvath B, Patsatsi Α, Uzun S, Bech R, Beissert S, et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV). J Eur Acad Dermatol Venereol. 2020;34(9):1900-1913. PubMed PMID: 32830877
  26. Joly P, Murrell DF, Aoyama Y, Caux F, De D, Flouri M, et al. Efficacy and safety of subcutaneous efgartigimod with prednisone in moderate-to-severe pemphigus (ADDRESS): a global, phase III, randomised controlled, double-blind trial. Br J Dermatol. 2026;195(5):750-762. PubMed PMID: 42723554
  27. Kridin K, Zelber-Sagi S, Bergman R. Pemphigus Vulgaris and Pemphigus Foliaceus: Differences in Epidemiology and Mortality. Acta Derm Venereol. 2017;97(9):1095-1099. PubMed PMID: 28536732
  28. Cai SC, Zhao X, Tang MB, Lim YL. Epidemiology and Factors Associated with Remission of Pemphigus Vulgaris and Foliaceus in Singapore. Ann Acad Med Singap. 2020;49(6):367-376. PubMed PMID: 32712634
  29. Baican A, Chiorean R, Leucuta DC, Baican C, Danescu S, Ciuce D, et al. Prediction of survival for patients with pemphigus vulgaris and pemphigus foliaceus: a retrospective cohort study. Orphanet J Rare Dis. 2015;10:48. PubMed PMID: 25896794

Label source: US prescribing information for rituximab, DailyMed (sections 1.5, 2.7, 14.8 and the boxed warning), DailyMed: rituximab.

PubMed Topic Searches

  1. Pemphigus foliaceus
  2. Fogo selvagem (endemic pemphigus foliaceus)
  3. Pemphigus foliaceus and rituximab
  4. Desmoglein 1 in pemphigus foliaceus
  5. Drug-induced pemphigus

Connections