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BMC Veterinary Research Nov 2020Pemphigus is the term used to describe a group of rare mucocutaneous autoimmune bullous diseases characterized by flaccid blisters and erosions of the mucous membranes... (Review)
Review
Pemphigus is the term used to describe a group of rare mucocutaneous autoimmune bullous diseases characterized by flaccid blisters and erosions of the mucous membranes and/or skin. When the autoantibodies target desmosomes in the deep layers of the epidermis, deep pemphigus variants such as pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus develop. In this article, we will review the signalment, clinical signs, histopathology and treatment outcome of pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus in dogs, cats and horses; where pertinent, we compare the animal diseases to their human homologue. Canine, feline and equine pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus have many features similar to the human counterpart. These chronic and often relapsing autoimmune dermatoses require aggressive immunosuppressive therapy. In animals, the partial-to-complete remission of pemphigus vulgaris and pemphigus vegetans has been achieved with high dose glucocorticoid therapy, with or without adjunct immunosuppressants; the prognosis is grave for paraneoplastic pemphigus.
Topics: Animal Diseases; Animals; Cats; Dogs; Horses; Immunosuppressive Agents; Pemphigus
PubMed: 33228633
DOI: 10.1186/s12917-020-02677-w -
Hua Xi Kou Qiang Yi Xue Za Zhi = Huaxi... Dec 2021Drug-induced pemphigus (DIP) is a special type of pemphigus, and its pathogenesis, characteristics of treatment, and prognosis are closely related to the inducing drugs....
Drug-induced pemphigus (DIP) is a special type of pemphigus, and its pathogenesis, characteristics of treatment, and prognosis are closely related to the inducing drugs. This article reports the diagnosis and treatment of DIP (pemphigus vulgaris) caused by the administration of rifampin to a patient with tuberculosis. Combined with the literature, we discussed the types, pathogenesis, differential diagnosis, and treatment principles of DIP. We propose that in the oral clinical practice for patients with pemphigus vulgaris, the importance of investigating suspected drugs that induce DIP should be emphasized.
Topics: Diagnosis, Differential; Humans; Pemphigus; Pharmaceutical Preparations; Prognosis
PubMed: 34859635
DOI: 10.7518/hxkq.2021.06.016 -
Lancet (London, England) Aug 1999Pemphigus and bullous pemphigoid are distinct autoimmune blistering diseases that are characterised by the presence of autoantibodies directed against specific adhesion... (Review)
Review
Pemphigus and bullous pemphigoid are distinct autoimmune blistering diseases that are characterised by the presence of autoantibodies directed against specific adhesion molecules of the skin and mucous membranes. The comparison and contrast of molecular mechanism of blister formation of these two diseases provide a rational diagnostic and therapeutic approach to affected patients.
Topics: Autoantibodies; Humans; Paraneoplastic Syndromes; Pemphigoid, Bullous; Pemphigus
PubMed: 10466686
DOI: 10.1016/S0140-6736(99)03007-X -
Dermatology Online Journal Aug 2020Pemphigus has been associated with other autoimmune and autoinflammatory disorders. Specifically, some case reports in the literature document coexistence of pemphigus... (Meta-Analysis)
Meta-Analysis
Pemphigus has been associated with other autoimmune and autoinflammatory disorders. Specifically, some case reports in the literature document coexistence of pemphigus with psoriasis, but this association is lacking larger scale investigation. With this in mind, we conducted a systematic review and meta-analysis to evaluate the association between pemphigus and psoriasis. In doing so, we found an association between the two conditions. Pemphigus was more common in patients with psoriasis than in controls (OR 2.64, 95% CI 1.24-5.59, P=0.01), with heterogeneity (I2=94%). We go on to propose pathophysiologic mechanisms and its relevance for diagnostic and management considerations.
Topics: Humans; Pemphigus; Psoriasis
PubMed: 32941727
DOI: No ID Found -
BMJ Case Reports May 2020
Topics: Adult; Female; Glucocorticoids; Humans; Mouth Diseases; Pemphigus; Prednisolone
PubMed: 32404374
DOI: 10.1136/bcr-2020-235410 -
Oral Diseases Oct 2023Autoimmune diseases (ADs) affect about 5% of the general population, causing various systemic and/or topical clinical manifestations. The oral mucosa is often affected,... (Review)
Review
Autoimmune diseases (ADs) affect about 5% of the general population, causing various systemic and/or topical clinical manifestations. The oral mucosa is often affected, sometimes as the only involved site. The misdiagnosis of oral ADs is an underreported issue. This narrative review focuses on diagnostic delay (DD) in oral ADs (oral lichen planus [OLP], oral Pemphigus Vulgaris, mucous membrane pemphigoid, oral lupus erythematosus, orofacial granulomatosis, oral erythema multiforme [EM], and Sjogren syndrome). Extensive literature research was conducted via MEDLINE, Embase and Google Scholar databases for articles reporting the time spent to achieve the correct diagnosis of oral ADs. Only 16 studies reported DD in oral ADs. Oral autoimmune vesiculobullous diseases are usually diagnosed after 8 months from the initial signs/symptoms, the Sjogren Syndrome diagnosis usually requires about 73 months. No data exist about the DD in OLP, oral lupus erythematosus, orofacial granulomatosis, and oral EM. The diagnosis of oral ADs can be difficult due to the non-specificity of their manifestations and the unawareness of dentists, physicians, and dental and medical specialists about these diseases. This can lead to a professional DD and a consequential treatment delay. The delay can be attributed to the physicians or/and the healthcare system (Professional Delay) or the patient (Patient's Delay).
Topics: Humans; Delayed Diagnosis; Sjogren's Syndrome; Granulomatosis, Orofacial; Autoimmune Diseases; Mouth Diseases; Pemphigus; Lupus Erythematosus, Systemic; Lichen Planus, Oral
PubMed: 36565434
DOI: 10.1111/odi.14480 -
Indian Journal of Dermatology,... 2011Pemphigus, bullous pemphigoid, and epidermolysis bullosa acquisita are autoimmune diseases of skin associated with considerable morbidity and sometimes mortality. There... (Review)
Review
BACKGROUND
Pemphigus, bullous pemphigoid, and epidermolysis bullosa acquisita are autoimmune diseases of skin associated with considerable morbidity and sometimes mortality. There is no cure for these diseases.
AIMS
To summarize evidence-based treatments for these diseases by performing a systematic review.
METHODS
The research protocol included the following steps: identification of databases to be searched, defining search strategy, searching the databases for references, first-stage screening of the abstracts, second-stage screening of full texts of articles identified after the first-stage screening, data extraction from the identified articles after second-stage screening, quality appraisal of the studies using the Delphi list, and summarizing the findings.
RESULTS
No randomized controlled trials of interventions in pemphigus vegetans, pemphigus erythematosus, and epidermolysis bullosa acquisita could be found. After the second-stage screening, 12 randomized controlled trials were analyzed, which included patients with pemphigus vulgaris or pemphigus vulgaris and pemphigus foliaceus, and 7 which included patients with bullous pemphigoid.
CONCLUSIONS
Number of high-quality randomized controlled trials conducted on pemphigus and bullous pemphigoid is small. Oral corticosteroid along with a steroid-sparing agent appears to be the most effective treatment for pemphigus. Azathioprine may be most effective as a steroid-sparing agent. Topical corticosteroid therapy (as studied) is effective for bullous pemphigoid and appears to be superior to oral corticosteroid for extensive disease. Some suggestions about future research are made.
Topics: Animals; Evidence-Based Medicine; Humans; Immunosuppressive Agents; Pemphigoid, Bullous; Pemphigus; Randomized Controlled Trials as Topic
PubMed: 21727693
DOI: 10.4103/0378-6323.82400 -
Anais Brasileiros de Dermatologia 2014The pemphigus group comprises the autoimmune intraepidermal blistering diseases classically divided into two major types: pemphigus vulgaris and pemphigus foliaceous.... (Review)
Review
The pemphigus group comprises the autoimmune intraepidermal blistering diseases classically divided into two major types: pemphigus vulgaris and pemphigus foliaceous. Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms that present some clinical, histological and immunopathological characteristics that are different from the classical types. These are reviewed in this article. Future research may help definitively to locate the position of these forms in the pemphigus group, especially with regard to pemphigus herpetiformis and the IgG/ IgA pemphigus.
Topics: Female; Humans; Immunoglobulin A; Immunoglobulin G; Male; Paraneoplastic Syndromes; Pemphigus; Rare Diseases; Skin
PubMed: 24626654
DOI: 10.1590/abd1806-4841.20142459 -
Acta Dermato-venereologica Nov 2019Pemphigus is an autoimmune skin disease characterized by blistering and erosions of the skin and mucous membranes. Pemphigus is rare in Northern and Western Europe but...
Pemphigus is an autoimmune skin disease characterized by blistering and erosions of the skin and mucous membranes. Pemphigus is rare in Northern and Western Europe but its incidence is higher around the Mediterranean Sea. The most common type worldwide is pemphigus vulgaris. The aim of this study was to investigate the incidence of pemphigus subtypes in Northern Finland between 1985 and 2017. A total of 46 patients diagnosed with pemphigus at the Department of Dermatology of Oulu University Hospital were found the female/male ratio was 1.7. In contrast to many other countries it was found that in Northern Finland the superficial pemphigus subtypes were the most common: erythematosus or foliaceus (65%) followed by pemphigus vulgaris (26%). Over the past 4 decades the annual incidence of pemphigus in Finland has increased from 0.76 to 2.8 cases per million persons.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Child, Preschool; Erythema; Female; Finland; Humans; Incidence; Infant; Infant, Newborn; Male; Middle Aged; Pemphigus; Sex Distribution; Time Factors; Young Adult
PubMed: 31396635
DOI: 10.2340/00015555-3280 -
Minerva Stomatologica Oct 2009Pemphigus is a group of potentially life-threatening autoimmune diseases characterized by cutaneous and/or mucosal blistering, due to the presence circulating IgG...
Pemphigus is a group of potentially life-threatening autoimmune diseases characterized by cutaneous and/or mucosal blistering, due to the presence circulating IgG antibodies directed against desmoglein 1 and 3 (Dsg 1 and 3). Differences in the particular distribution of these result in different clinical manifestations of the disease. The most common variant is pemphigus vulgaris (PV). There is a fairly strong genetic background to pemphigus with linkage to HLA class II alleles and ethnic groups such as Ashkenazi Jews and those of Mediterra-nean and Indian origin are especially liable. Oral lesions are commonly characterized by the presence of vesiculobullous and ulcerative lesions. Diagnosis is achieved via three different parameters: perilesional tissue biopsy, histological and immunological examinations. Serum autoantibodies to either Dsg1 or Dsg3 are best detected using both normal human skin and monkey esophagus or by enzyme-linked immunosorbent assay. The main aim of treatment is to reduce inflammatory re-sponse and autoantibody production, in order to achieve disease remission in a short time. Before the advent of corticosteroids, PV was typically fatal due to dehydration or secondary systemic infections. Current treatment is largely based on systemic immunosuppression using corticosteroids, with azathioprine or other adjuvants or alternatives. Nonetheless, newer therapies, such as intravenous immunoglobulins (IVIg) or anti-CD20 monoclonal antibodies (Rituximab), with potentially fewer adverse effects also appear promising.
Topics: Humans; Mouth Diseases; Pemphigus
PubMed: 19893475
DOI: No ID Found