Materiały źródłowe

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

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

  Pemphigus vulgaris is an uncommon, potentially fatal, autoimmune disorder characterized by intraepidermal blisters and extensive erosions on apparently healthy skin and mucous membranes. 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.

• #2 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.

• #3 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. […] Patients often see five or more providers before receiving a correct diagnosis. […] In October 2011, KJT Group was commissioned by the IPPF to conduct an awareness and diagnostic pathways survey. […] On average, it took patients 10 months to achieve a correct diagnosis. […] Another study, published in December 2000 by Dr. David Sirois, et al.* surveyed 99 PV patients and found that 80% of patients experienced oral lesions as the first sign of PV, and 24% of patients only experienced oral lesions.

• #4 Pemphigus Vulgaris: A Deep Dive into Diagnosis – BLDG Active

  https://www.bldgactive.com/blog-pemphigus-vulgaris-a-deep-dive-into-diagnosis/?srsltid=AfmBOopR1oKxlVRj0HhvX7-JP3ceVWUB6HRgr9HxFt3WU_lTIKVCgwBG

  Pemphigus Vulgaris, a rare autoimmune blistering disorder, can be a challenging condition to diagnose. […] Early diagnosis of Pemphigus Vulgaris is crucial. Delayed diagnosis can result in the progression of the disease, making treatment less effective and potentially causing life-threatening complications. […] Diagnosing Pemphigus Vulgaris begins with recognizing its symptoms. […] Diagnosing Pemphigus Vulgaris involves a multi-step process: […] A dermatologist or specialist will conduct a thorough examination of the patient’s skin, mouth, and mucous membranes. […] A skin or mucous membrane biopsy is often necessary. This involves taking a small sample of affected tissue for examination under a microscope. In Pemphigus Vulgaris, a biopsy typically reveals specific patterns of cell separation.

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

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

  Pemphigus vulgaris is an uncommon, potentially fatal, autoimmune disorder characterized by intraepidermal blisters and extensive erosions on apparently healthy skin and mucous membranes. 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.

• #6 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. […] Patients often see five or more providers before receiving a correct diagnosis. […] In October 2011, KJT Group was commissioned by the IPPF to conduct an awareness and diagnostic pathways survey. […] On average, it took patients 10 months to achieve a correct diagnosis. […] Another study, published in December 2000 by Dr. David Sirois, et al.* surveyed 99 PV patients and found that 80% of patients experienced oral lesions as the first sign of PV, and 24% of patients only experienced oral lesions.

• #7 Pemphigus – Symptoms, diagnosis and treatment | BMJ Best Practice US

  https://bestpractice.bmj.com/topics/en-us/454

  Pemphigus vulgaris (PV) is the most common variant and affects skin and mucosa. […] Diagnosis is based on clinical findings of erosions (epidermal loss) and blisters of the skin, mucosa, or both. […] Direct immunofluorescence staining of biopsied skin shows immunoglobulin and C3 deposits on the keratinocyte surface, and histology reveals keratinocyte acantholysis. […] Key diagnostic factors include chronic erosive blistering of the skin, mucosa, or both, chronic mouth erosions (PV, PNP), painful lips (PNP), and shortness of breath (PNP). […] 1st tests to order include skin biopsy, hematoxylin and eosin stain and skin biopsy, direct immunofluorescence. […] Tests to consider include indirect immunofluorescence on serum, serum ELISA, upper gastrointestinal endoscopy, CXR, chest CT scan, PFT, and serum immunoblot (Western blot).

• #8 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.

• #9 Pemphigus vulgaris: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/000882.htm

  The skin separates easily when the surface of unaffected skin is rubbed sideways with a cotton swab or finger. This is called a positive Nikolsky sign. […] A skin biopsy and blood tests are often done to confirm the diagnosis.

• #10 What’s Pemphigus? What Causes It?

  https://www.webmd.com/skin-problems-and-treatments/what-is-pemphigus

  Pemphigus Diagnosis […] It can be tricky. Thats because a number of conditions can cause blisters. To make sure they find the right cause, your doctor will likely order a number of tests, including: […] Medical history. Your doctor will ask you what medications you take, as some drugs can cause this condition. […] A skin exam. Your doctor will use their finger or a cotton swab to rub a patch of your skin thats not covered by a blister. If it peels easily, you could have pemphigus. […] Skin biopsy. Your doctor will take a piece of tissue from one of your blisters and look at it under a microscope. […] Blood tests. Your doctor will check your blood for specific antibodies called desmogleins. Antibodies are proteins your body makes to find bad germs and kill them before they harm you.

• #11 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.

• #12 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.

• #13 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.

• #14 Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment | IntechOpen

  https://www.intechopen.com/chapters/57959

  Pemphigus is a group of autoimmune blistering disorders associated with autoantibodies against the keratinocyte cell surface. […] Together with clinical manifestations, the histopathological and immunopathological data support the diagnosis. […] The best site for the cutaneous biopsy for the appropriate histopathological examination is a fresh (24 h) small vesicle or 1/3 of the peripheral portion of the blister including the perilesional normal appearing skin. […] For direct immunofluorescence microscopic (DIF) examination, a perilesional normal appearing skin area up to 1 cm from a fresh vesicle should be taken and should be transformed in saline or in a cylinder of liquid nitrogen in a period lesser than 36 h. […] As though some pemphigus variants, particularly PV and PNP, are potentially life-threatening diseases, early diagnosis is necessary and early onset of immunosuppressive treatment should be promptly initiated.

• #15 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.

• #16 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.

• #17 Pemphigus Vulgaris: A Complete Overview — DermNet

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

  How is pemphigus vulgaris diagnosed? 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. See pathology of pemphigus vulgaris. […] 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 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.

• #19 Pemphigus Vulgaris: A Complete Overview — DermNet

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

  How is pemphigus vulgaris diagnosed? 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. See pathology of pemphigus vulgaris. […] 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.

• #20 Pemphigus Laboratory Testing | Beutner Labs

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

  Pemphigus vulgaris may also present clinically as pemphigus vegitans. […] Direct immunofluorescence (DIF) is 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%. […] Biopsy studies of perilesional skin or mucosa by direct IF are considered a gold standard for the diagnosis of pemphigus. […] Circulating antibodies in pemphigus vulgaris can be detected by two serum tests: Indirect immunofluorescence (IIF) tests for IgG and IgG4 intercellular antibodies/cell surface antibodies. […] Sensitivity of Dsg3 ELISA for pemphigus vulgaris is 100% and specificity 99.2%.

• #21 SciELO Brazil – Pemphigus vulgaris Pemphigus vulgaris

  https://www.scielo.br/j/abd/a/nV5V8fJtZw7kQhhDbzffyVL/

  Cytological examination can be used for the rapid demonstration of acantholytic keratinocytes of the spinous layer. […] Histopathological examination helps to identify the level of blister cleavage in order to diagnose pemphigus, and to differentiate with other subepidermal bullous lesions. […] Direct immunofluorescence examination is considered by some authors as a gold standard for the differential diagnosis of PV. […] The identification of IgG and C3 autoantibodies directed against the cell surface of keratinocytes is considered by some authors as a gold standard for the differential diagnosis of PV. […] The most sensitive and specific test to confirm the diagnosis of pemphigus vulgaris is direct immunofluorescence. […] For the laboratory diagnosis of PV, Tzanck smear, histopathological examination, direct immunofluorescence examination, or even immunohistochemical examination may be used.

• #22 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.

• #23 Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment | IntechOpen

  https://www.intechopen.com/chapters/57959

  Pemphigus is a group of autoimmune blistering disorders associated with autoantibodies against the keratinocyte cell surface. […] Together with clinical manifestations, the histopathological and immunopathological data support the diagnosis. […] The best site for the cutaneous biopsy for the appropriate histopathological examination is a fresh (24 h) small vesicle or 1/3 of the peripheral portion of the blister including the perilesional normal appearing skin. […] For direct immunofluorescence microscopic (DIF) examination, a perilesional normal appearing skin area up to 1 cm from a fresh vesicle should be taken and should be transformed in saline or in a cylinder of liquid nitrogen in a period lesser than 36 h. […] As though some pemphigus variants, particularly PV and PNP, are potentially life-threatening diseases, early diagnosis is necessary and early onset of immunosuppressive treatment should be promptly initiated.

• #24 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.

• #25 Pemphigus vulgaris

  https://www.pcds.org.uk/clinical-guidance/pemphigus-vulgaris

  Pemphigus vulgaris is a rare immunobullous condition that is characterised by blisters and erosions on the skin and mucous membranes, most commonly the mouth. […] In pemphigus vulgaris IgG autoantibodies are directed to the pemphigus vulgaris antigen, also known as desmoglein-3, which is found in desmosomes in the keratinocytes near the bottom of the epidermis. […] A request for skin antibodies covers both pemphigoid and pemphigus antibodies. In pemphigus vulgaris, circulating pemphigus antibodies are detected in over 80% of cases. […] Two skin biopsies are required. An intact blister should be excised and sent for histology. A second biopsy, of peri-lesional skin (within 2 cm of the blister), is also required for direct immunofluorescence (DIF). […] Histology shows suprabasal clefts and blisters, containing some acantholytic cells. […] DIF shows IgG deposited on the surface of keratinocytes throughout the epidermis.

• #26 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.

• #27 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.

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

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

  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). […] ELISA testing is positive in more than 95% of PV cases. […] An alternative test is indirect immunofluorescence (IIF) microscopy, in which anti-epithelial cell surface IgG deposits can be detected on the epithelium of monkey or guinea pig esophagus or human skin when exposed to serum from a PV patient.

• #29 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.

• #30 Pemphigus | Choose the Right Test

  https://arupconsult.com/content/pemphigus

  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.

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

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

  Pemphigus vulgaris is an uncommon, potentially fatal, autoimmune disorder characterized by intraepidermal blisters and extensive erosions on apparently healthy skin and mucous membranes. 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.

• #32 Pemphigus Laboratory Testing | Beutner Labs

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

  Pemphigus vulgaris may also present clinically as pemphigus vegitans. […] Direct immunofluorescence (DIF) is 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%. […] Biopsy studies of perilesional skin or mucosa by direct IF are considered a gold standard for the diagnosis of pemphigus. […] Circulating antibodies in pemphigus vulgaris can be detected by two serum tests: Indirect immunofluorescence (IIF) tests for IgG and IgG4 intercellular antibodies/cell surface antibodies. […] Sensitivity of Dsg3 ELISA for pemphigus vulgaris is 100% and specificity 99.2%.

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

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

  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). […] ELISA testing is positive in more than 95% of PV cases. […] An alternative test is indirect immunofluorescence (IIF) microscopy, in which anti-epithelial cell surface IgG deposits can be detected on the epithelium of monkey or guinea pig esophagus or human skin when exposed to serum from a PV patient.

• #34 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. […] The changes made to previous guidelines are summarized in the Supplemental Table I. […] The laboratory work-up is delineated in Table I. […] The persistence of high levels of anti-Dsg1 by ELISA has a positive predictive value for skin relapses, whereas the persistence of anti-Dsg3 IgG does not necessarily indicate a mucosal relapse. […] Discontinuation of treatment is primarily based on the clinical symptoms but may also be supported by the findings of Dsg ELISA, IIF microscopy, and/or a negative result of DIF microscopy of a skin biopsy specimen.

• #35 Pemphigus vulgaris* | Anais Brasileiros de Dermatologia

  http://www.anaisdedermatologia.org.br/en-pemphigus-vulgaris-articulo-S0365059620306048

  Direct immunofluorescence examination showing moderate intensity for the IgG and C3 markers, with intercellular fluorescence distribution, often with predominant location in the lower layers of the epithelium. […] Indirect immunofluorescence assists in the diagnosis of PV and allows the detection of circulating autoantibodies. […] ELISA is a very sensitive and specific method that allows detection of IgG anti-Dsg1 and anti-Dsg3 autoantibodies in over 90% of patients using recombinant Dsg1 and Dsg3.

• #36 Pemphigus vulgaris* | Anais Brasileiros de Dermatologia

  http://www.anaisdedermatologia.org.br/en-pemphigus-vulgaris-articulo-S0365059620306048

  Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. […] Clinical suspicion can be confirmed by cytological examination, histopathological examination, and direct and indirect immunofluorescence tests. […] For the laboratory diagnosis of PV, Tzanck smear, histopathological examination, direct immunofluorescence examination, or even immunohistochemical examination may be used. […] Cytological examination (Tzanck smear) is useful for the rapid demonstration of acantholytic keratinocytes of the spinous layer. […] Histopathological examination helps to identify the level of blister cleavage in order to diagnose pemphigus, and to differentiate with other subepidermal bullous lesions. […] The identification of IgG and C3 autoantibodies directed against the cell surface of keratinocytes is considered by some authors as a „gold standard” for the differential diagnosis of PV.

• #37 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. […] In the physical examination, the characteristics and extent of skin and mucous membrane lesions, and the degree of mucosal damage and functional impairment is assessed. […] Laboratory assessments are then undertaken using skin biopsies and blood samples to confirm that it is PV and not another blistering or erosive disease. […] These assessments include detecting active acantholysis (histopathology), autoantibodies at the surface of epidermal keratinocytes (direct immunofluorescence microscopy) and circulating autoantibodies (serology).

• #38 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.

• #39 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. […] PV is treatable and prompt intervention may prevent the development of more severe symptoms.

• #40 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.

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

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

  Pemphigus vulgaris is an uncommon, potentially fatal, autoimmune disorder characterized by intraepidermal blisters and extensive erosions on apparently healthy skin and mucous membranes. 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.

• #42 Pemphigus Vulgaris: A Complete Overview — DermNet

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

  How is pemphigus vulgaris diagnosed? 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. See pathology of pemphigus vulgaris. […] 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.

• #43 Bullous Pemphigoid & Pemphigus Vulgaris- Dermatology Advisor

  https://www.dermatologyadvisor.com/ddi/bullous-pemphigoid-pemphigus-vulgaris/

  The diagnosis of PV is accomplished through a combination of clinical findings, histopathology, and immunohistochemistry. Diagnosis is confirmed by DIF assay, indirect immunofluorescence (IIF) assay, or ELISA to Dsg1 and Dsg3 recombinant fusion proteins. DIF reveals deposition of IgG and C3 binding to the keratinocyte cell surface in the mid and lower or entire epidermis of the perilesional skin or mucosa. An ELISA kit for detection and titration of circulating anti-Dsg3 and anti-Dsg1 antibodies has recently become available to aid in diagnosis. […] However, despite the multiple tools available, there are currently no uniformly accepted criteria for the diagnosis of PV. […] The differential diagnosis for PV includes BP, pemphigus foliaceus, pemphigus herpetiformis, dermatitis herpetiformis, and IgA pemphigus. BP is characterized by pruritic, tense blisters localized to the flexural aspects of the extremities and the trunk. Unlike that seen in PV, most patients with BP do not suffer from oral involvement and they will have a negative Nikolsky sign.

• #44 Orphanet: Pemphigus vulgaris

  https://www.orpha.net/en/disease/detail/704

  Diagnosis should be considered in the presence of bullae on chest or scalp. It can be confirmed by standard histopathological analysis and direct immunofluorescence (DIF) test. Intraepidermal blisters due to suprabasal acantholysis and IgG (mostly IgG1 and IgG4) and/or C3 complement deposits can be detected at the cell surface of keratinocytes. The disease grade is correlated with the level of circulating antibodies. […] Differential diagnosis includes the full range of subepidermal diseases, mainly mucous membrane pemphigoid and epidermolysis bullosa acquisita. Oral erosive lichen planus and recurrent buccal aphtosis are characterized by a negative DIF pattern.

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

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

  Pemphigus vulgaris is an uncommon, potentially fatal, autoimmune disorder characterized by intraepidermal blisters and extensive erosions on apparently healthy skin and mucous membranes. 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.

• #46 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. […] The changes made to previous guidelines are summarized in the Supplemental Table I. […] The laboratory work-up is delineated in Table I. […] The persistence of high levels of anti-Dsg1 by ELISA has a positive predictive value for skin relapses, whereas the persistence of anti-Dsg3 IgG does not necessarily indicate a mucosal relapse. […] Discontinuation of treatment is primarily based on the clinical symptoms but may also be supported by the findings of Dsg ELISA, IIF microscopy, and/or a negative result of DIF microscopy of a skin biopsy specimen.

• #47 Pemphigus | Choose the Right Test

  https://arupconsult.com/content/pemphigus

  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.

• #48 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. […] The changes made to previous guidelines are summarized in the Supplemental Table I. […] The laboratory work-up is delineated in Table I. […] The persistence of high levels of anti-Dsg1 by ELISA has a positive predictive value for skin relapses, whereas the persistence of anti-Dsg3 IgG does not necessarily indicate a mucosal relapse. […] Discontinuation of treatment is primarily based on the clinical symptoms but may also be supported by the findings of Dsg ELISA, IIF microscopy, and/or a negative result of DIF microscopy of a skin biopsy specimen.

• #49 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.

• #50 Pemphigus Vulgaris: A Deep Dive into Diagnosis – BLDG Active

  https://www.bldgactive.com/blog-pemphigus-vulgaris-a-deep-dive-into-diagnosis/?srsltid=AfmBOopR1oKxlVRj0HhvX7-JP3ceVWUB6HRgr9HxFt3WU_lTIKVCgwBG

  Pemphigus Vulgaris, a rare autoimmune blistering disorder, can be a challenging condition to diagnose. […] Early diagnosis of Pemphigus Vulgaris is crucial. Delayed diagnosis can result in the progression of the disease, making treatment less effective and potentially causing life-threatening complications. […] Diagnosing Pemphigus Vulgaris begins with recognizing its symptoms. […] Diagnosing Pemphigus Vulgaris involves a multi-step process: […] A dermatologist or specialist will conduct a thorough examination of the patient’s skin, mouth, and mucous membranes. […] A skin or mucous membrane biopsy is often necessary. This involves taking a small sample of affected tissue for examination under a microscope. In Pemphigus Vulgaris, a biopsy typically reveals specific patterns of cell separation.

• #51 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

  Pemphigus vulgaris (PV) is a chronic autoimmune bullous disease caused by autoantibodies to proteins in the oral mucosa and skin. […] Early diagnosis is important because the disease has a high mortality rate if untreated appropriately in the early stages, and rapid treatment initiation is associated with rapid disease control. […] The diagnosis is based on the identification of clinical manifestations and confirmed through biopsy. Direct immunofluorescence is employed for the conclusive diagnosis of PV. […] 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. […] Clinicians should be aware that early diagnosis is difficult in cases of PV in which oral lesions are the only symptom. […] 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.

• #52 Pemphigus: Diagnosis and treatment

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

  This could lead to better treatment for pemphigus vulgaris. […] Today, the outcome looks good. For most people, the disease can be controlled with treatment. Many can eventually stop their treatment for a while. […] Before medicines like prednisone and azathioprine were used to treat pemphigus, a person lived about 5 years after getting pemphigus vulgaris, the most common type. Thanks to medicines and other treatments, this has changed. Few people die of pemphigus. […] These problems can be managed with help from your dermatologist and other doctors.

• #53 Pemphigus: Diagnosis and treatment

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

  Blood test: This test can tell whether you have proteins in your blood that cause pemphigus. […] If you are diagnosed with pemphigus, you’ll likely need treatment. Without treatment, pemphigus tends to worsen. In some people, pemphigus can become life-threatening without treatment. […] Pemphigus cannot be cured, but with treatment, most people can control their pemphigus. […] Treatment can reduce (and sometimes clear) the blisters and sores caused by all types of pemphigus. Treatment can also prevent pemphigus from worsening. […] A treatment plan for pemphigus may include one or more of the following: […] Corticosteroid: If you have mild pemphigus, your dermatologist may prescribe a corticosteroid that you apply to your skin. Many people need stronger medicine like prednisone or methylprednisolone. These corticosteroids work throughout the body.

• #54 Pemphigus: Diagnosis and treatment

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

  A corticosteroid can clear the blisters and sores. […] Immunosuppressant medication: This medication quiets (or suppresses) the immune system. Either azathioprine or mycophenalate mofetil is often used to treat pemphigus. These can stop the body from creating new blisters. […] Biologics: This is a newer treatment option. One biologic, rituximab, seems promising. It appears to offer safe treatment. Because pemphigus is rare, we don’t have the studies needed to know who will respond and what long-term side effects are possible. […] Researchers are looking for better treatment options. […] One area of research shows promise. At the University of Pennsylvania School of Medicine, scientists have found a way to successfully remove the cells that cause pemphigus vulgaris. They have successfully treated mice using this technique, without harming healthy tissue.

• #55 Pemphigus vulgaris* | Anais Brasileiros de Dermatologia

  http://www.anaisdedermatologia.org.br/en-pemphigus-vulgaris-articulo-S0365059620306048

  Direct immunofluorescence examination showing moderate intensity for the IgG and C3 markers, with intercellular fluorescence distribution, often with predominant location in the lower layers of the epithelium. […] Indirect immunofluorescence assists in the diagnosis of PV and allows the detection of circulating autoantibodies. […] ELISA is a very sensitive and specific method that allows detection of IgG anti-Dsg1 and anti-Dsg3 autoantibodies in over 90% of patients using recombinant Dsg1 and Dsg3.

• #56 Pemphigus vulgaris* | Anais Brasileiros de Dermatologia

  http://www.anaisdedermatologia.org.br/en-pemphigus-vulgaris-articulo-S0365059620306048

• #57 Pemphigus: Diagnosis and treatment

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

  Blood test: This test can tell whether you have proteins in your blood that cause pemphigus. […] If you are diagnosed with pemphigus, you’ll likely need treatment. Without treatment, pemphigus tends to worsen. In some people, pemphigus can become life-threatening without treatment. […] Pemphigus cannot be cured, but with treatment, most people can control their pemphigus. […] Treatment can reduce (and sometimes clear) the blisters and sores caused by all types of pemphigus. Treatment can also prevent pemphigus from worsening. […] A treatment plan for pemphigus may include one or more of the following: […] Corticosteroid: If you have mild pemphigus, your dermatologist may prescribe a corticosteroid that you apply to your skin. Many people need stronger medicine like prednisone or methylprednisolone. These corticosteroids work throughout the body.

• #58 Pemphigus vulgaris

  https://www.nhs.uk/conditions/pemphigus-vulgaris/

  Pemphigus vulgaris can have similar symptoms to more common conditions, such as impetigo and hand, foot and mouth disease. […] If a GP thinks you could have pemphigus vulgaris, they can refer you to a skin specialist (dermatologist) for tests and treatment. […] Blisters caused by pemphigus vulgaris usually come and go over time. There’s no cure, but treatment can help. […] It usually takes a few weeks for the blisters to heal. Once this happens, you’ll probably need treatment for several years to help stop the blisters coming back. […] If the blisters come back, you may need to repeat treatment.

• #59

  https://dpcj.org/index.php/dpc/article/view/dermatol-pract-concept-articleid-dp1003a50

  Autoimmune bullous disorders are a heterogeneous spectrum of skin disorders characterized by the production of autoantibodies against adhesion molecules of the skin. […] Diagnosis is based on clinical manifestations and confirmed with histological, immunofluorescence, and serological testing. […] Recently multivariant enzymelinked immunosorbent assay systems have been developed as practical screening tools for patients with suspected autoimmune bullous dermatoses.

• #60 Her Strange Blisters Wouldn’t Go Away. What Was It? – The New York Times

  https://www.nytimes.com/2023/10/07/magazine/pemphigus-vulgaris-diagnosis.html

  The P.A. explained that bullous pemphigoid (B.P.) is a rare autoimmune disease in which the body’s white blood cells create antibodies that attack the connection between the skin and the tissue below, causing these blisters. […] “You have pemphigus vulgaris,” Alloo told her, “and you are going to get better.” Pemphigus gets its name from the Greek word for blister. […] Until recently the diagnosis was made solely by biopsy. Now a blood test can help identify the specific antibodies that do the damage.

• #61 Pemphigus: Diagnosis and treatment

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

  A corticosteroid can clear the blisters and sores. […] Immunosuppressant medication: This medication quiets (or suppresses) the immune system. Either azathioprine or mycophenalate mofetil is often used to treat pemphigus. These can stop the body from creating new blisters. […] Biologics: This is a newer treatment option. One biologic, rituximab, seems promising. It appears to offer safe treatment. Because pemphigus is rare, we don’t have the studies needed to know who will respond and what long-term side effects are possible. […] Researchers are looking for better treatment options. […] One area of research shows promise. At the University of Pennsylvania School of Medicine, scientists have found a way to successfully remove the cells that cause pemphigus vulgaris. They have successfully treated mice using this technique, without harming healthy tissue.

• #62 2025 ICD-10-CM Diagnosis Code L10.0: Pemphigus vulgaris

  https://www.icd10data.com/ICD10CM/Codes/L00-L99/L10-L14/L10-/L10.0

  L10.0 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. […] An autoimmune blistering disorder. It is characterized by the presence of painful blisters and erosions in the skin and mucous membranes. […] ICD-10-CM L10.0 is grouped within Diagnostic Related Group(s) (MS-DRG v42.0): 595 Major skin disorders with mcc, 596 Major skin disorders without mcc. […] Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.

• #63 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. […] The changes made to previous guidelines are summarized in the Supplemental Table I. […] The laboratory work-up is delineated in Table I. […] The persistence of high levels of anti-Dsg1 by ELISA has a positive predictive value for skin relapses, whereas the persistence of anti-Dsg3 IgG does not necessarily indicate a mucosal relapse. […] Discontinuation of treatment is primarily based on the clinical symptoms but may also be supported by the findings of Dsg ELISA, IIF microscopy, and/or a negative result of DIF microscopy of a skin biopsy specimen.

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

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

  In summary, here we have presented the recommendations arising from a Delphi process involving 39 pemphigus experts. We have made recommendations for evaluation and treatment of pemphigus, including initial evaluation, diagnosis, and management, as well as regarding strategies for maintenance therapy and tapering of medications in remission.

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