Materiały źródłowe

• #1 Diagnosis – IPPF

  https://www.pemphigus.org/diagnosis/

  Early diagnosis may permit successful treatment with only low levels of medication. Consult a dermatologist or oral medicine specialist if there are any persistent skin or mouth lesions. Because they are so rare, pemphigus and pemphigoid are often the last disease considered during diagnosis. […] For a definite diagnosis, doctors should consider: Clinical presentation visual examination of skin or oral lesions. Lesion biopsy a sample of the blistered skin is removed and examined under the microscope. Additionally, the layer of skin in which cell-to-cell separation occurs can be determined. Direct immunofluorescence the skin sample is treated to detect desmoglein antibodies in the skin. The presence of these antibodies indicates pemphigus. Indirect Immunofluorescence or antibody titer test. This measures desmoglein autoantibodies in the blood serum. It may be used to obtain a more complete understanding of the course of the disease. ELISA A serum assay for desmoglein antibodies, known as ELISA, is also available. Although in many cases there is a correlation between ELISA and disease activity it is not so in every case.

• #1 Diagnosis – IPPF

  https://www.pemphigus.org/diagnosis/

  A biopsy and immunofluorescent studies are needed to achieve a correct diagnosis. […] Dentists considering PV/MMP in their differential diagnosis should perform a biopsy or make a referral to a dental specialist experienced in performing biopsies of vesiculobullous lesions. Unfortunately, patients are not always referred to the appropriate provider, delaying diagnosis. […] Delayed diagnosis of PV/MMP may: Increase patient pain and suffering, Delay treatment, Increase a patients risk for disease progression to other sites, Make it more difficult for a patient to achieve remission, Heighten risk for poor treatment response, Result in loss of productivity and reduced quality of life, Cause uncertainty, fear, and anxiety, Result in severe weight loss, Increase patient healthcare expenses. […] On average, it took patients 10 months to achieve a correct diagnosis. More than half of patients reported extreme difficulty in finding a doctor knowledgeable enough to accurately diagnose (56%) and effectively treat (51.2%) their condition.

• #2 Diagnostics – IPPF

  https://www.pemphigus.org/diagnostics/

  Early diagnosis may permit successful treatment with only low levels of medication. Consult a dermatologist or oral medicine specialist if there are any persistent skin or mouth lesions. Because they are so rare, pemphigus and pemphigoid are often the last disease considered during diagnosis. […] For a definite diagnosis, doctors should consider: Clinical presentation: visual examination of skin or oral lesions. Lesion biopsy: A sample of the blistered skin is removed and examined under the microscope. Additionally, the layer of skin in which cell-to-cell separation occurs can be determined. Direct immunofluorescence: The skin sample is treated to detect desmoglein antibodies in the skin. The presence of these antibodies indicates pemphigus. Indirect Immunofluorescence or antibody titer test: This measures desmoglein autoantibodies in the blood serum. It may be used to obtain a more complete understanding of the course of the disease. ELISA: A serum assay for desmoglein antibodies, known as ELISA, is also available. Although in many cases there is a correlation between ELISA and disease activity it is not so in every case.

• #2 Diagnostics – IPPF

  https://www.pemphigus.org/diagnostics/

  Diagnosis of all bullous conditions is based on three measures: Clinical features: appearance of lesions, erosions and blisters, Nikolsky sign, presence or absence of scarring and distribution of lesions. Biopsy of skin or mucous membrane, with characteristic histological features. Direct and indirect immunofluorescence: detection of autoantibodies either in a biopsy specimen (direct) or in the patients serum (indirect). Indirect (circulating) antibodies are helpful in confirming the diagnosis. The enzyme-linked immunoabsorbent assay (ELISA) is helpful for diagnosis, particularly if the immunofluorescence studies are negative, and to differentiate PV from PF; in the future newer more specific ELISAs hold promise as a prognostic indicator. […] Accurate diagnosis of oral blistering diseases is predicated on obtaining a satisfactory biopsy for both hematoxylin and eosin (HE) and direct immunofluorescence (DIF) evaluation. To be diagnostic, the biopsy specimens must contain intact epithelium over the underlying connective tissue. However, in the oral cavity these lesions are often fragile and separation between the epithelium and connective tissue is common, rendering the specimen non-diagnostic. A biopsy should be performed by a dentist or dental specialist experienced in performing biopsies of vesiculobullous lesions.

• #3 Pemphigus – Wikipedia

  https://en.wikipedia.org/wiki/Pemphigus

  Pemphigus defines a group of autoimmune intraepithelial blistering diseases that are characterized by loss of normal cell-cell adhesion (acantholysis), and by the presence of pathogenic (predominantly IgG) autoantibodies reacting against epithelial adhesion molecules. […] Definitive diagnosis requires examination of a skin or mucous membrane biopsy by a dermatopathologist or oral pathologist. The skin biopsy is taken from the edge of a blister, prepared for histopathology and examined with a microscope. The pathologist looks for an intraepidermal vesicle caused by the breaking apart of epidermal cells (acantholysis). Thus, the superficial (upper) portion of the epidermis sloughs off, leaving the bottom layer of cells on the „floor” of the blister. This bottom layer of cells is said to have a „tombstone” appearance.

• #4 Diagnosis and management of pemphigus: Recommendations of an international panel of experts

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7313440/

  Several European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management. […] The diagnosis of pemphigus is based on the following criteria: Clinical presentation, Histopathology, Direct immunofluorescence (DIF) microscopy of perilesional skin, Serologic detection of serum autoantibodies against epithelial cell surface by indirect immunofluorescence (IIF) microscopy and/or enzyme-linked immunosorbent assay (ELISA). […] Diagnosis requires clinical presentation and histopathology that are consistent with pemphigus and either a positive DIF microscopy or serologic detection of autoantibodies against epithelial cell surface antigens. […] The initial evaluation of suspected pemphigus should seek to determine the signs or symptoms present that would corroborate the diagnosis of pemphigus, as well as to screen for possible comorbidities.

• #4 Diagnosis and management of pemphigus: Recommendations of an international panel of experts

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7313440/

  The management of patients with pemphigus is the responsibility of dermatologists with experience in treating bullous diseases. […] The overall disease management is coordinated by the dermatologist with the cooperation of the referring dermatologist/family practitioner, the general physician, and other medical specialists and hospital doctors from the center of reference and/or geographic area (if a reference center exists in the particular country). […] The changes made to previous guidelines are summarized in the Supplemental Table I. […] The laboratory work-up is delineated in Table I. […] The first round of surveys was delivered via email in February 2016 and completed by 39 expert participants. […] The results of the survey were tallied and delivered to the group. […] A median score of 70 percent or greater per question was used as the consensus threshold for agreement, and a median score of 30 or lower was established as the consensus threshold for disagreement.

• #5 Pemphigus | Choose the Right Test

  https://arupconsult.com/content/pemphigus

  Pemphigus refers to a group of uncommon and severe autoimmune blistering skin diseases that affect the epithelium, including skin and mucous membranes. […] Because pemphigus is rare and has features that mimic other more common disorders, diagnosis can be challenging and often delayed. Diagnosis is based on clinical characteristics, histopathologic features, and the detection of disease-specific autoantibodies in tissue and/or serum using direct immunofluorescence (DIF) microscopy, indirect immunofluorescence (IIF) testing (also referred to as indirect immunofluorescence, or IIF), and enzyme-linked immunosorbent assays (ELISAs). […] Testing for pemphigus is appropriate in patients with chronic, recurring bullae (blisters), erosive or crusting/scaling skin, or mucous membrane disease that is not attributable to a more common cutaneous disorder.

• #5 Pemphigus | Choose the Right Test

  https://arupconsult.com/content/pemphigus

  In addition to clinical and histopathologic features, diagnosis of pemphigus depends on detection of immunoglobulin G (IgG) and/or complement component 3 (C3) on the membranes of keratinocytes with DIF microscopy of perilesional tissue, along with detection of antibodies against desmoglein 1 and/or desmoglein 3 in serum. […] Broad epithelial antibody screening is generally recommended due to the overlapping clinical presentations among immunobullous skin diseases. […] IIF tests and ELISAs have utility in monitoring disease activity and response to therapy, given that antibody levels reflect disease activity in the most common forms of pemphigus.

• #6 Pemphigus – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pemphigus/diagnosis-treatment/drc-20350409

  Your healthcare professional may start by talking with you about your medical history and symptoms and examining the affected area. In addition, you may have tests, including: […] A biopsy is a procedure to remove a sample of tissue for testing in a lab. To test for pemphigus, a piece of the blister is used. […] One purpose of these tests is to detect and identify antibodies in your blood that are known to be present with pemphigus. […] Your healthcare professional may refer you to a specialist in skin conditions. This type of specialist is a dermatologist. […] For pemphigus, some basic questions to ask your healthcare professional include: Do I need any tests? Do these tests require any special preparation? […] Did you have a skin biopsy?

• #7 Pemphigus: Diagnosis, Treatment, and Steps to Take

  https://www.niams.nih.gov/health-topics/pemphigus/diagnosis-treatment-and-steps-to-take

  Early diagnosis is important, so if you have blisters on the skin or in the mouth that do not go away, it is important to see a doctor as soon as you can. […] Your doctor may try to rule out other conditions first, since pemphigus is a rare disease. […] A dermatologist (a doctor who specializes in conditions of the skin, hair, and nails) may ask you about your medical history and look at the appearance and location of blisters. […] He or she may run a finger or cotton swab over the surface of your skin to see if it shears off easily. […] Your doctor may take a sample from one of your blisters to: Examine it under the microscope to look for cell separation and to determine the layer of skin in which the cells are separated. […] Determine which antibodies attacked the skin. […] Blood tests can help determine the types of antibodies that are in the blood and their levels, which can help predict the severity of the disease. […] This blood test may also be used later on to see if treatment is working.

• #8 Pemphigus: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/pemphigus-treatment

  Dermatologists are the experts in skin disease, so they are often asked by other doctors to examine a patient who could have pemphigus. Many different skin diseases cause blistering, and an accurate diagnosis is so important. […] If a dermatologist suspects you may have pemphigus, you’ll likely need the following: […] Physical exam: Your dermatologist will look at the blisters, sores, and crusts. If you have blisters on your skin, your dermatologist will lightly rub a bit of normal-looking skin near a blister to find out if the skin comes off. […] Medical history: Your dermatologist will ask questions about your health and what medicines you take. […] Skin biopsy: Your dermatologist can easily perform a skin biopsy during the office visit. To do this, your dermatologist will remove part of a new blister and some normal-looking skin (or other tissue) from near the blister. These will be looked at under a microscope in a lab.

• #9 Pemphigus Vulgaris Workup: Laboratory Studies, Histologic Findings

  https://emedicine.medscape.com/article/1064187-workup

  In the patient’s serum, IDIF demonstrates the presence of circulating IgG autoantibodies that bind to epidermis. Circulating intercellular antibodies are detected by means of IDIF in 80-90% of patients with pemphigus vulgaris. The titer of circulating antibody correlates with disease course. […] Histopathologic evaluation demonstrates an intraepidermal blister. The earliest changes consist of intercellular edema with loss of intercellular attachments in the basal layer. Suprabasal epidermal cells separate from the basal cells to form clefts and blisters. Basal cells are separated from one another and stand like a row of tombstones on the floor of the blister, but they remain attached to the basement membrane. Blister cells contain some acantholytic cells. Histopathology can help differentiate pemphigus vulgaris from pemphigus foliaceus, which demonstrates a more superficial epidermal cleavage.

• #9 Pemphigus Vulgaris Workup: Laboratory Studies, Histologic Findings

  https://emedicine.medscape.com/article/1064187-workup

  To establish a diagnosis of pemphigus vulgaris, the following tests are performed: Histopathology from the edge of a blister […] Direct immunofluorescence (DIF) on normal-appearing perilesional skin […] Indirect immunofluorescence (IDIF) using the patient’s serum if DIF results are positive – The preferred substrate for IDIF is monkey esophagus or salt-split normal human skin substrate. The best location for DIF testing is on normal perilesional skin. When DIF testing is performed on lesional skin, false-positive results can be observed. Skin biopsy specimens placed in transport media may yield false-negative results; therefore, fresh tissue is the preferred substrate for DIF studies. DIF demonstrates in vivo deposits of antibodies and other immunoreactants, such as complement. DIF usually shows immunoglobulin G (IgG) deposited on the surface of the keratinocytes in and around lesions; IgG1 and IgG4 are the most common subclasses. Complement components such as C3 and immunoglobulin M are present less frequently than IgG. DIF shows intercellular deposition throughout the epidermis. This pattern of immunoreactants is not specific for pemphigus vulgaris and may be seen in pemphigus vegetans, pemphigus foliaceus, and pemphigus erythematosus.

• #10 Pemphigus Foliaceus – Dermatologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/dermatologic-disorders/bullous-diseases/pemphigus-foliaceus

  Pemphigus foliaceus is an autoimmune blistering disorder in which lack of adhesion in the superficial epidermis results in cutaneous erosions. Diagnosis is by skin biopsy and direct immunofluorescence. […] Diagnosis of pemphigus foliaceus is by biopsy of a lesion and adjacent (perilesional) unaffected skin that shows IgG autoantibodies against the keratinocyte cell surface via direct immunofluorescence. Autoantibodies to desmoglein 1 can be detected in serum via direct immunofluorescence, indirect immunofluorescence, and enzyme-linked immunosorbent assay (ELISA). Sensitivity of ELISA is 95% (1). […] Diagnose using skin biopsy and direct immunofluorescence.

• #11 Pemphigus Erythematosus – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/pemphigus-erythematosus/

  In 1926, Francis Senear and Barney Usher described 11 patients with features of a pemphigus-lupus erythematosus overlap, which they named pemphigus erythematosus, later to be known as Senear-Usher syndrome. […] Walter Lever observed that some patients previously diagnosed as having pemphigus erythematosus in fact had systemic lupus erythematosus, or went on to develop more widespread pemphigus foliaceus (PF), or even pemphigus vulgaris, in many cases due to an incorrect initial diagnosis. […] Pemphigus erythematosus will show acantholysis (loss of intercellular adhesion between intact keratinocytes) in the granular or subcorneal layer of the epidermis, identical to the blister of pemphigus foliaceus. […] Autoantibodies to the keratinocyte cell surface should be documented, either by direct immunofluorescence, indirect immunofluorescence, or ELISA.

• #11 Pemphigus Erythematosus – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/pemphigus-erythematosus/

  By definition, all pemphigus erythematosus patients will have the cell surface immunofluorescence staining pattern characteristic of pemphigus foliaceus. […] Serum sample for indirect immunofluorescence (IIF). Serum is incubated with epithelial substrates (typically guinea pig esophagus or normal human skin). […] Indirect immunofluorescence is a semi-quantitative test, with titer roughly correlating with disease activity. […] Desmoglein 1 ELISA is more sensitive and specific (100% and 96%, respectively) for PF than indirect immunofluorescence and may replace the latter as the preferred diagnostic test. […] Immunologic testing for lupus should be performed. […] The clinical differential diagnosis for pemphigus erythematosus, or localized PF, can include bullous impetigo, seborrheic dermatitis, rosacea, herpes simplex, or eczema.

• #12 A Diagnostic Approach to Pemphigus and Pemphigoid – The Dermatology Digest

  https://thedermdigest.com/a-diagnostic-approach-to-pemphigus-and-pemphigoid/

  We use immunofluorescence not only in the diagnosis of autoimmune mucocutaneous blistering disorders like pemphigus and pemphigoid, but we can also use it the diagnosis of autoimmune connective tissue diseases, vasculitis, vasculopathy, porphyria, pseudoporphyria, and lichenoid dermatoses, said Alina G. Bridges, DO, FAAD, who presented Pemphigus and Pemphigoid Disorders in the session entitled A Practical Approach to Direct Immunofluorescence at the 2023 American Academy of Dermatology (AAD) Annual Meeting. […] The big overview on using immunofluorescence to diagnose these diseases is that you have to correlate the clinical presentation with the histopathology and then confirm the diagnosis by the immunofluorescence testing, which can be tissue-based or serum-based. Direct immunofluorescence is a tissue-based assay.

• #12 A Diagnostic Approach to Pemphigus and Pemphigoid – The Dermatology Digest

  https://thedermdigest.com/a-diagnostic-approach-to-pemphigus-and-pemphigoid/

  And as such, immunofluorescence testing is recommended, said Dr. Bridges. […] The direct immunofluorescence is a skin biopsy thats done in the office and thats where errors can occur because sometimes clinicians dont know where to biopsy correctly to diagnose pemphigus and pemphigoid. […] In this case, you have to biopsy uninvolved or perilesional skin, and you have to know how to submit the biopsy because you cant put it in formalin. […] Next, serum-based testing using indirect immunofluorescence and ELISA antibody testing is recommended to confirm the diagnosis, said Dr. Bridges. […] It is important that we go beyond direct immunofluorescence testing, she said. […] You need to consider these diagnoses in your differential diagnosis especially in atypical presentations and know how to appropriately evaluate these patients to establish the diagnosis so that the diagnosis is not delayed and so that you can appropriately manage the patient.

• #12 A Diagnostic Approach to Pemphigus and Pemphigoid – The Dermatology Digest

  https://thedermdigest.com/a-diagnostic-approach-to-pemphigus-and-pemphigoid/

  The longer these conditions remain undiagnosed, the longer it can take to get them under control and the higher the initial antibody titers at the time of diagnosis. […] Serum-based testing not only plays a role in diagnosis but plays a role is pathogenesis, disease monitoring, prognosis, and response to therapy. […] According to Dr. Bridges, it can take years of experience to recognize when to consider these diseases in your clinical and histopathological differential diagnosis and the type of workup that needs to be performed. […] I would say that the greatest pitfall is to not do immunofluorescence testing.

• #13 Pemphigus Laboratory Testing | Beutner Labs

  https://www.beutnerlabs.com/pemphigus-laboratory-testing

  Pemphigus refers to a group of rare, potentially lethal acantholytic autoimmune diseases affecting skin and mucous membranes. […] Due to the significant morbidity and mortality associated with this group of autoimmune blistering diseases, an accurate diagnostic work-up is imperative for management. […] Confirmatory DIF and serology studies are indicated. Histologic studies reveal intraepidermal or intraepithelial, acantholytic bullae. […] Biopsy studies of perilesional skin or mucosa by direct IF are considered a gold standard for the diagnosis of pemphigus. […] The sensitivity of DIF for pemphigus ranges from 90%-100% and the specificity and the positive predictive value of this test for pemphigus is 100%. […] Circulating antibodies in pemphigus vulgaris can be detected by two serum tests: […] Sensitivity of Dsg1 ELISA for pemphigus foliaceus is reported to be 96.0% and specificity 98.6%. Sensitivity of Dsg3 ELISA for pemphigus vulgaris is 100% and specificity 99.2%. […] Other forms can be distinguished by diagnostic laboratory studies.

• #14 Pemphigus Vulgaris – Dermatologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/dermatologic-disorders/bullous-diseases/pemphigus-vulgaris

  Diagnosis is by skin biopsy with direct and indirect immunofluorescence and enzyme-linked immunosorbent assay (ELISA) testing. […] Pemphigus vulgaris should be suspected in patients with unexplained chronic mucosal ulceration, particularly if they have bullous skin lesions. This disorder must be differentiated from other disorders that cause chronic oral ulcers and from other bullous dermatoses (eg, pemphigus foliaceus, bullous pemphigoid, mucous membrane pemphigoid, drug eruptions, toxic epidermal necrolysis, erythema multiforme, dermatitis herpetiformis, bullous contact dermatitis). […] The diagnosis of pemphigus vulgaris is confirmed by biopsy of lesional and surrounding (perilesional) normal skin. Immunofluorescence testing shows IgG autoantibodies against the keratinocyte’s cell surface. Serum autoantibodies to desmoglein 1 and desmoglein 3 transmembrane glycoproteins can be identified via direct immunofluorescence, indirect immunofluorescence, and enzyme-linked immunosorbent assay (ELISA). Sensitivity of ELISA is higher ( 95% than that of indirect immunofluorescence. […] Use Nikolsky and Asboe-Hansen signs to help clinically differentiate pemphigus vulgaris from other bullous disorders. […] Confirm the diagnosis by immunofluorescence testing of skin samples.

• #15 IgA Pemphigus Workup: Laboratory Studies, Other Tests, Procedures

  https://emedicine.medscape.com/article/1063776-workup

  Skin biopsy is performed in IgA pemphigus (see Procedures). […] Perform other tests, if available, to document the diagnosis of IgA pemphigus, including immunoblotting, enzyme-linked immunosorbent assay (ELISA), and special immunofluorescence studies. […] Immunoblotting documents the specific skin antigen recognized by the patient’s IgA autoantibodies. […] Immunoblotting has shown a low sensitivity rate for showing IgA reactivity to any autoantigen (40%). […] In theory, ELISA should be a good methodology for documenting the specific desmosomal antigen(s) recognized by patient IgA autoantibodies, since it uses native protein and increases detection sensitivity. […] ELISAs have shown a low sensitivity rate for showing IgA reactivity to any autoantigen (55%). […] Special immunofluorescence tests using cultured cells that express recombinant desmosomal component (desmocollin 1) document the presence of circulating IgA autoantibodies that recognize a desmosomal component.

• #16 Pemphigus Vulgaris (PV) | Diagnosis, Treatment & Management

  https://medically.roche.com/global/en/microsites/about-pv/pv-treatment-management.html

  Rapid and accurate diagnosis of PV is needed to ensure patients receive appropriate treatment early. […] Diagnosis of PV involves a clinical examination (medical history and physical examination) and a series of laboratory investigations to confirm PV. […] Laboratory assessments are then undertaken using skin biopsies and blood samples to confirm that it is PV and not another blistering or erosive disease. […] A small biopsy (3-5 mm punch excision) of affected skin (blister and/or lesion) is taken and examined for evidence of acantholysis in the basal layer by histopathology. […] A skin biopsy (1 cm of fresh lesion) is examined for the presence of IgG autoantibodies to DSG3 and DSG1 and possible complement C3 protein deposits on the surface of epidermal keratinocytes. […] B-cell produced anti-DSG1 and/or anti-DSG3 antibodies are measured in serum of patients with PV by enzyme-linked immunosorbent assay (ELISA).

• #16 Pemphigus Vulgaris (PV) | Diagnosis, Treatment & Management

  https://medically.roche.com/global/en/microsites/about-pv/pv-treatment-management.html

  PV may be first seen by general practitioners, dentists, oral surgeons or gynecologists, depending on where the symptoms first appear. Rapid referral to a specialist dermatologist is important to ensure early diagnosis. […] The primary objective of PV treatment is to control and heal the bullous skin and/or mucosal lesions, whilst minimizing the side effects of treatment. […] For the therapeutic management of PV, the guideline provides recommendations for initial and maintenance treatment for mild and moderate to severe PV. […] The consensus group also provides recommendations on supportive treatment, such as proper dental care, intralesional injections of corticosteroids, topical treatment with potent corticosteroids, antiseptic baths, analgesics, local anesthetics and nutritional management, and prophylaxis against side effects in prolonged corticosteroid therapy.

• #17 Pemphigus Vulgaris: A Complete Overview — DermNet

  https://dermnetnz.org/topics/pemphigus-vulgaris

  Diagnosis of pemphigus vulgaris generally requires a biopsy of a blister. Diagnosis of pemphigus vulgaris generally requires a biopsy from the skin adjacent to a lesion. Histology typically shows rounded-up and separated keratinocytes (acantholytic cells) just above the basal layer of the epidermis. Suprabasal clefting may be reported. […] Pemphigus is confirmed by direct immunofluorescence staining of perilesional skin biopsy sections to reveal immunoglobulin (Ig)G antibodies or complement on the cell surfaces of keratinocytes. […] In most cases, circulating antibodies can be detected by a blood test (indirect immunofluorescence test). The level of antibodies fluctuates and may reflect the effectiveness of treatment. Specific anti-dsg1 and anti-dsg3 antibody titres can also be measured in blood or saliva by enzyme-linked immunosorbent assays (ELISAs). […] Pemphigus vulgaris may co-exist with or be confused with pemphigus foliaceus, cicatricial pemphigoid and lichen planus.

• #18 Pemphigus: Types, Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/21130-pemphigus

  Pemphigus is a group of autoimmune skin conditions that cause sores, blisters or fluid-filled bumps to form on your skin and mucus membranes. […] Pemphigus is sometimes confused with other autoimmune blistering skin conditions such as bullous pemphigoid, lupus erythematosus and Hailey-Hailey disease. […] Pemphigus can affect anyone. Its most common among people between the ages of 40 and 60. […] Pemphigus isnt common. An estimated 1 to 5 out of every 1 million people receive a pemphigus diagnosis throughout the world each year. […] Your healthcare provider will diagnose pemphigus after performing a physical exam, learning more about your medical history and offering tests that include: […] Theyll take a small sample of your skins tissue and examine it under a microscope. […] Theyll examine a sample of your blood to look for antibodies that cause the condition.

• #19 Pemphigus Vulgaris: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/23033-pemphigus-vulgaris

  Your healthcare provider will diagnose pemphigus vulgaris after a physical exam to learn more about your symptoms and will ask questions about your health and medical history. Theyll also offer tests to diagnose your condition. […] To confirm a diagnosis, your healthcare provider might offer a skin biopsy. During a skin biopsy, your healthcare provider will remove a small sample of your tissue from an affected area of your skin to examine it under a microscope. This may include an immunofluorescence test. […] Another test is a blood test to check for immune system antibodies that cause your symptoms.

• #20 Pemphigus Vulgaris – Dermatology – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.856.3.

  The diagnosis of PV is based on the combination of clinical presentation, histologic findings, direct immunofluorescence findings, and detection of circulating autoantibodies. […] Circulating autoantibodies can be detected by indirect immunofluorescence (antiskin antibodies in pemphigus) in ~80% to 90% of patients with PV. […] There is a new technique, enzyme-linked immunosorbent assay (ELISA) for antibodies against Dsg1 and Dsg3, which is more specific and more sensitive and can be useful in monitoring the disease activity. […] Two skin biopsies should be performed when PV is suspected. […] Direct immunofluorescence of the perilesional skin is the gold standard to detect tissue-bound autoantibodies.

• #21 Pemphigus Vulgaris: Diagnosis, Treatment, and Prognosis

  https://ostrowonline.usc.edu/pemphigus-vulgaris-diagnosis-treatment-and-prognosis/

  Where do we find them in patients? […] Early diagnosis of Pemphigus Vulgaris is something we try to achieve in patients because the oral lesions are the first show and last to go. […] If you can catch and make the diagnosis early, you can potentially prevent skin lesions, which is why Pemphigus Vulgaris is something that you need to have on your radar. […] So if you are suspecting Pemphigus Vulgaris, you need to take a biopsy. For biopsies of vesicular ulcerative conditions, you want to include perilesional tissue and the lesional tissue. This is really really important. We need to see the separation where the vesicle is forming in order to make the diagnosis.

• #22 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Pemphigus-Foliaceus.aspx

  Pemphigus foliaceus is usually diagnosed by skin biopsy. […] Diagnosis is confirmed using direct immunofluorescence staining, which reveals the presence of the autoantibodies that attack epidermis cells. […] Sometimes, a blood test may be used to detect antibodies in the circulation.

• #23 IgA pemphigus

  https://dermnetnz.org/topics/iga-pemphigus

  A blistering autoimmune disease or autoinflammatory pustular disease may be suspected clinically from the clinical features, often after the eruption has failed to clear with antibiotics. […] The diagnosis of IgA pemphigus requires a skin biopsy for histology and direct immunofluorescent testing. […] In IgA pemphigus, a skin biopsy reveals intraepidermal neutrophil infiltration. Blistering skin specimens will show mixed inflammatory infiltrate in upper, mid or lower epidermis, depending on the subtype with little or no acantholysis. […] Direct immunofluorescence of a skin biopsy of healthy appearing skin close to the area of blistering reveals IgA deposition in epidermal keratinocyte cell membranes. […] Indirect immunofluorescence testing of serum can show IgA anti-keratinocyte cell-surface antibodies. Immunoglobulin G (IgG) anti-keratinocyte cell-surface antibodies have also been reported.

• #24 Paraneoplastic Pemphigus | Choose the Right Test

  https://arupconsult.com/content/paraneoplastic-pemphigus

  Paraneoplastic pemphigus, also referred to as paraneoplastic autoimmune multiorgan syndrome, is a severely debilitating blistering disease that affects skin and mucous membranes and nearly always involves an underlying neoplasm. […] Diagnosis can be challenging because of the tendency of paraneoplastic pemphigus to mimic other cutaneous diseases. Accurate diagnosis requires assessment of clinical characteristics and histopathologic features, along with the detection of disease-specific autoantibodies in tissue and/or serum using methodologies such as direct immunofluorescence (DIF) microscopy, indirect immunofluorescence (IIF) testing, and enzyme-linked immunosorbent assays (ELISAs). […] Strong clinical suspicion for paraneoplastic pemphigus in the absence of known cancer should prompt a thorough evaluation for possible malignancy.

• #24 Paraneoplastic Pemphigus | Choose the Right Test

  https://arupconsult.com/content/paraneoplastic-pemphigus

  Diagnosis of paraneoplastic pemphigus is based on supportive histopathologic findings on examination of formalin-fixed tissue, immunopathologic features, with demonstration on DIF microscopy of characteristic immunoglobulin G (IgG) and/or complement component 3 (C3) reactivity patterns, detection of autoantibodies to epithelial components in serum, and compatible clinical features. […] DIF microscopy, used to detect tissue-bound autoantibodies, is performed on perilesional skin biopsy tissue and is an important part of an evaluation for paraneoplastic pemphigus. […] In addition to tissue evaluation using DIF microscopy, assessment for pemphigus involves detection and identification of circulating autoantibodies in serum. […] Available serum tests include IIF testing and ELISAs. […] If an autoantibody screen yields positive results in patients without a known malignancy, an aggressive evaluation for malignancy is warranted. […] Testing for pemphigus is appropriate in patients with suspected malignancy (a neoplasm typically precedes skin disease) or cutaneous manifestations that are consistent with paraneoplastic pemphigus and are not attributable to a more common cutaneous disorder.

• #25 SSA – POMS: DI 23022.635 – Paraneoplastic Pemphigus – 10/06/2023

  https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022635

  DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING […] Diagnostic testing: There is no single test to confirm a diagnosis of PNP. Because PNP is a rare disease of the skin, a dermatologist is consulted to diagnose and treat this disease. The dermatologist will document the appearance and location of the blisters. Direct immunofluorescence (on skin or mucosal biopsies) and indirect immunofluorescence (on blood) are done to identify the antibodies and the type of pemphigus causing the skin blisters. While a tumor is essential in the diagnosis, people with tumors other than lymphoproliferative neoplasm can develop paraneoplastic pemphigus. These include thymoma, sarcoma, and lung carcinoma. […] Laboratory testing reports documenting PNP serum antibody screen, and results of biopsies are needed to confirm the diagnosis.

• #26 A Sensitive Method for the Diagnosis of Pemphigus | CCID

  https://www.dovepress.com/direct-immunofluorescence-of-igg-on-formalin-fixed-paraffin-embedded-t-peer-reviewed-fulltext-article-CCID

  Direct immunofluorescence (DIF) on frozen sections (DIF-F) plays a key role in the identification and differential diagnosis of bullous dermatoses, which are a group of critical autoimmune diseases that include pemphigus, bullous pemphigoid (BP), and epidermolysis bullosa acquisita (EBA). […] In this study, the application value of DIF on paraffin-embedded tissue sections (DIF-P) detecting IgG using heat-induced antigen retrieval (HIAR) in the diagnosis of bullous dermatosis was explored. […] All patients were diagnosed with the autoimmune bullous disease (AIBD) based on clinical presentation, histopathology, DIF-F, and enzyme-linked immunosorbent assay (ELISA). […] The detection of IgG by DIF-P using HIAR can be used for the diagnosis of pemphigus as an alternative method to DIF-F. […] This study shows that DIF-P by the HIAR method can be used to confirm intercellular IgG deposition in pemphigus lesions.

• #26 A Sensitive Method for the Diagnosis of Pemphigus | CCID

  https://www.dovepress.com/direct-immunofluorescence-of-igg-on-formalin-fixed-paraffin-embedded-t-peer-reviewed-fulltext-article-CCID

  In summary, when there are no fresh frozen samples but paraffin-embedded tissues are available, DIF-P could be chosen as a reliable diagnostic method. […] In conclusion, this study found that DIF-P staining IgG using HIAR can be used as a diagnostic tool for pemphigus and has clinical significance as an alternative to DIF-F when it is not possible.

• #27 Academic Excellence | PUMCH Dermatology Leads Development of China’s First Pemphigus Diagnosis and Treatment Guideline – PUMCH – PUMCH KEYWORDS

  https://www.pumch.cn/en/detail/38413.html

  It recommends using the Pemphigus Disease Area Index (PDAI) to evaluate disease severity before and during treatment. […] The treatment approach has also been updated. […] For mild cases, corticosteroid therapy is recommended, with immunosuppressants added when necessary. […] For moderate to severe cases, the Guideline recommends combination therapy of corticosteroids with either rituximab or immunosuppressants. […] The Guideline provides detailed protocols for the use of corticosteroids, immunosuppressants and rituximab and addresses additional treatment options including intravenous immunoglobulin, plasma exchange, and immunoadsorption, aided by comprehensive treatment flowcharts. […] This Guideline marks a significant advance towards proper and more standardized pemphigus care in China. […] The department remains committed to advancing research and promoting consistent diagnosis and treatment to provide more therapeutic options for pemphigus patients.

• #28 Addressing Challenges in Diagnosis, Differential Diagnosis, and Treatment of Pemphigus: A Case Series

  https://www.mdpi.com/2075-4418/13/24/3633

  Pemphigus is a rare autoimmune disease characterized by skin blisters and erosions, with or without mucosal involvement. The clinical presentation of pemphigus can resemble other bullous diseases, leading to challenges in diagnosis. […] In conclusion, pemphigus may mimic other bullous diseases, making diagnosis challenging. A comprehensive clinical and laboratory assessment is necessary to provide accurate diagnosis and treatment. […] The diagnosis of pemphigus or other diseases that mimic pemphigus can be challenging for many reasons. In clinical practice, unusual or atypical manifestations of the bullous disease can be a diagnostic challenge, sometimes causing a delay in diagnosis, and so a variety of differential diagnoses depending on the clinical picture should always be considered. […] Histopathological examination can help to diagnose pemphigus and distinguish it from other sub-epidermal bullous lesions, as acantholysis keratinocytes can be seen in several vesiculobullous diseases.

• #29 Early Diagnosis and Management of Oral Pemphigus Vulgaris Lesions of Various Presentations

  https://www.journalomp.org/journal/view.html?doi=10.14476/jomp.2023.48.4.174

  Therefore, differentiating PV from other similar oral lesions based on the clinical appearance is extremely difficult. Clinical presentation, histopathology, immunofluorescence, and serologic tests should be considered when making a definitive diagnosis. […] Accurate early diagnosis is necessary to ensure that patients do not suffer from delayed diagnosis and accompanying unnecessary treatment and that appropriate initial therapy can be started as soon as needed.

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