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Medicina (Kaunas, Lithuania) Oct 2021Clinicians may encounter a variety of skin conditions that present with vesiculobullous lesions in their everyday practice. Pemphigus vulgaris, pemphigus foliaceus, IgA... (Review)
Review
Clinicians may encounter a variety of skin conditions that present with vesiculobullous lesions in their everyday practice. Pemphigus vulgaris, pemphigus foliaceus, IgA pemphigus, and paraneoplastic pemphigus represent the spectrum of autoimmune bullous dermatoses of the pemphigus family. The pemphigus family of diseases is characterized by significant morbidity and mortality. Considering the risks associated with a delayed diagnosis or misdiagnosis and the potential for overlap in clinical features and treatment, evaluation for suspected pemphigus disease often requires thorough clinical assessment and laboratory testing. Diagnosis is focused on individual biopsies for histopathology and direct immunofluorescence. Additional laboratory methods used for diagnosis include indirect immunofluorescence and enzyme-linked immunosorbent assay. Recent advancements, including anti-CD20 therapy, have improved the efficacy and reduced the morbidity of pemphigus treatment. This contribution presents updates on the pathophysiology, clinical features, diagnostic work-up, and medical management of pemphigus. Improved strategies for diagnosis and clinical assessment are reviewed, and newer treatment options are discussed.
Topics: Autoimmune Diseases; Enzyme-Linked Immunosorbent Assay; Fluorescent Antibody Technique, Indirect; Humans; Pemphigus; Skin Diseases, Vesiculobullous
PubMed: 34684117
DOI: 10.3390/medicina57101080 -
Immunologic Research Apr 2018Pemphigus forms a group of rare autoimmune bullous diseases that affect the skin and mucous membranes. This group has a chronic course leading to high morbidity and... (Review)
Review
Pemphigus forms a group of rare autoimmune bullous diseases that affect the skin and mucous membranes. This group has a chronic course leading to high morbidity and mortality. It is characterized by the production of pathogenic autoantibodies directed against different proteins of the desmosome, leading histologically to intraepidermal cleavage, and clinically to vesicles and erosions on the epithelium of the mucous membranes and/or the skin. The diagnosis of the subtype of pemphigus is based on clinical features, the level of histologic cleavage, and the identification of the antigens recognized by circulating autoantibodies by immunoserological analyses. The epidemiological features of pemphigus vary considerably in different regions of the world. Observational studies examining comorbidities and associations among patients with pemphigus are scarce and sometimes inconclusive. The prognosis, mortality, and clinical outcomes in pemphigus have undergone dramatic change throughout the years. This review provides a brief overview about the different subtypes of pemphigus: pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, pemphigus herpetiformis, and IgA pemphigus. In addition, it summarizes the most recent understanding of the epidemiology, mortality data, and comorbidities of this group of organ-specific autoimmune diseases.
Topics: Autoantibodies; Humans; Pemphigus; Prognosis
PubMed: 29479654
DOI: 10.1007/s12026-018-8986-7 -
Journal of the American Academy of... Jun 1988The term pemphigus refers to a group of autoimmune intraepidermal blistering diseases of the skin and mucous membranes. Several clinical variants of pemphigus are... (Review)
Review
The term pemphigus refers to a group of autoimmune intraepidermal blistering diseases of the skin and mucous membranes. Several clinical variants of pemphigus are recognized. The major histologic feature of all variants is acantholysis, the disruption of normal cell-to-cell adhesion, which leads to intraepidermal blister formation. Most patients with pemphigus demonstrate IgG autoantibodies directed against an antigen located on the surface of keratinocytes. Although the stimulus for autoantibody production is unknown, several mechanisms have been proposed to explain the pathogenesis of acantholysis. One popular model proposes that pemphigus antibodies induce acantholysis through local stimulation of the plasminogen-plasmin system. Another model proposes that pemphigus antibodies fix complement and thereby alter cell membrane integrity to produce acantholysis. Prior to the availability of corticosteroids, pemphigus vulgaris was commonly fatal. Treatment with glucocorticosteroids has drastically improved the prognosis. Immunosuppressive agents and plasmapheresis have been used successfully in some patients with severe disease.
Topics: Humans; Pemphigus
PubMed: 3290286
DOI: 10.1016/s0190-9622(88)70128-0 -
European Journal of Dermatology : EJD Dec 2021The entity "contact pemphigus" has been recognized for more than 50 years, however existence of the disease, which is opposed and supported by many, is questionable.... (Review)
Review
The entity "contact pemphigus" has been recognized for more than 50 years, however existence of the disease, which is opposed and supported by many, is questionable. Contact pemphigus is defined as pemphigus occurring at the site of local skin contact with different chemicals. Many products have been disclosed as aetiological factors such as pesticides, topical drugs (imiquimod, ketoprofen, phenol, bezoin, polymyxin B sulphate, neomycin and bacitracin), cosmetics, garlic and others. This paper summarizes the current knowledge on contact pemphigus and the chemicals responsible for its aetiology, with an emphasis on mechanisms that may elicit the disease.
Topics: Humans; Pemphigus
PubMed: 33337331
DOI: 10.1684/ejd.2020.3916 -
The Veterinary Clinics of North... Jan 2019Feline pemphigus foliaceus is an uncommon skin disorder in cats but still the most common autoimmune skin disorder seen in this species. It is a crusting dermatosis... (Review)
Review
Feline pemphigus foliaceus is an uncommon skin disorder in cats but still the most common autoimmune skin disorder seen in this species. It is a crusting dermatosis involving the ears, head, paws, and ungual folds primarily. Although cytology is helpful in supporting a diagnosis, biopsy of pustules or crusts for histopathology is required for a definitive diagnosis. Therapy has evolved over the years as we have learned more about the different ways cats respond to drugs and as new drugs became available. Most cats will respond to proper therapy, although it may be needed long term for control.
Topics: Animals; Cat Diseases; Cats; Glucocorticoids; Immunosuppressive Agents; Pemphigus; Veterinary Medicine
PubMed: 30227972
DOI: 10.1016/j.cvsm.2018.08.009 -
Clinical and Experimental Dermatology Oct 2019Pemphigus diseases are cutaneous and mucous membrane blistering diseases, related to the key antigens of desmoglein 1 and 3. This article reviews the topic, including... (Review)
Review
Pemphigus diseases are cutaneous and mucous membrane blistering diseases, related to the key antigens of desmoglein 1 and 3. This article reviews the topic, including diagnosis, and provides the physician with guidance on the treatment of these difficult to control disorders.
Topics: Biopsy; Diagnosis, Differential; Fluorescent Antibody Technique, Direct; Humans; Maintenance Chemotherapy; Pemphigus; Practice Guidelines as Topic; Remission Induction; Skin
PubMed: 31378971
DOI: 10.1111/ced.14041 -
Lancet (London, England)Pemphigus is a rare autoimmune disease that results in blistering of the skin and oral cavity. It is caused by autoantibodies directed against cell-surface antigens on... (Review)
Review
Pemphigus is a rare autoimmune disease that results in blistering of the skin and oral cavity. It is caused by autoantibodies directed against cell-surface antigens on keratinocytes, which when targeted lose their cellular adhesion properties and separate from one another to form blisters within the epidermis. Differences in the particular antigens targeted by the antibodies and in the distribution of these antigens in the different regions of the body and in the separate layers of the epidermis result in different clinical manifestations of the disease. The disease is diagnosed based on its clinical manifestations (flaccid blisters and erosions on skin and oral mucosa), histology (epidermal acantholysis), and immunological abnormalities (circulating and tissue-fixed antibodies against keratinocyte surface antigens). Pemphigus, which if left untreated is almost always fatal, is generally managed with topical, oral, or intralesional corticosteroids. Other options include plasmapheresis and intravenous immunoglobulin (IVIg), coupled with cytotoxic drugs. Immunosupressants, anti-inflammatory drugs, and antibiotics are used as adjuvants, but apart from IVIg, these therapy options are non-specific and more research is needed to develop treatments with improved side-effect profiles.
Topics: Humans; Pemphigus
PubMed: 15993235
DOI: 10.1016/S0140-6736(05)66829-8 -
Actas Dermo-sifiliograficas 2005Pemphigus is an infrequent, organ-specific, autoimmune bullous disease, which affects the skin, mucous membranes and appendages. Histopathologically, it is characterized... (Review)
Review
Pemphigus is an infrequent, organ-specific, autoimmune bullous disease, which affects the skin, mucous membranes and appendages. Histopathologically, it is characterized by acantholysis. Pemphigus has classically been divided into two major groups, pemphigus vulgaris and pemphigus foliaceus, with their respective clinical variants pemphigus vegetans and pemphigus erythematosus. In recent years, new variants of pemphigus have been described: paraneoplastic pemphigus, IgA pemphigus and pemphigus herpetiformis. This article reviews the epidemiology, etiopathogenesis, clinical symptoms, diagnosis, treatment and prognosis of pemphigus. Advances in molecular biology techniques have made it possible to more precisely identify the different antigens against which antibodies are directed, and to fine-tune ELISA diagnostic techniques. Treating pemphigus vulgaris and foliaceus with general steroids has modified their prognosis; it is estimated that mortality in recent decades is less than 10 %. Managing the clinical complications that appear during the evolution of the pemphigus has contributed to reducing morbidity and mortality.
Topics: Humans; Paraneoplastic Syndromes; Pemphigus
PubMed: 16476253
DOI: 10.1016/s0001-7310(05)73090-8 -
Mayo Clinic Proceedings Apr 2024
Topics: Humans; Pemphigus
PubMed: 38569810
DOI: 10.1016/j.mayocp.2023.11.006 -
Journal of Cutaneous Medicine and... 2021
Topics: Adrenal Cortex Hormones; Diagnosis, Differential; Humans; Pemphigus; Prognosis
PubMed: 32602731
DOI: 10.1177/1203475420936656