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Epidemiology
Definition: Pemphigus is a life-threatening blistering disorder caused by a loss of keratinocyte to keratinocyte adhesion called acantholysis. This loss causes the eruption of intraepithelial blisters in the skin and the mucous membranes. The patients affected with pemphigus usually present mucosal erosions and/or pustules on the their skins. It is also an auto immune disease characterized by the adhesion of immunoglobulin G (IgG) autoantibodies to intercellular adhesion molecules (ICAMs). There are four major types of pemphigus: pemphigus vulgaris, pemphigus foliaceus, immunoglobulin A (IgA) pemphigus, and paraneoplastic pemphigus (Hertl & Sitaru, 2022).
Demographics: Even though, this disorder happens worldwide; however, the annual incidence rates between 0.1 and 0.5 per 100,000 people is mostly reported in individuals with Ashkenazi Jewish ancestry, and countries such as India, Southeast Europe, Middle East, North Africak,
Turkey, South America, and Brazil. Hertl and Sitaru (2022) stipulate: “Pemphigus usually occurs in adults, with an average age of onset of 40 to 60 years for pemphigus vulgaris and nonendemic pemphigus foliaceus” (para 8). Children are rarely affected but exceptionally cases of endemic pemphigus foliaceus were found in this population. The incidence is slightly more common in female. “A 4:1 ratio of females to males with pemphigus foliaceus in Tunisia, and a study that found a 19:1 ratio of males to females in an endemic location in Colombia” (Hertl & Sitaru, 2022, para 9).
Causative agent: The phenomenon of acantholysis is caused by the adhesion of autoantibodies especially IgA and IgG to the epithelial cell surface antigens.
Risk Factors & Exposures: Risk factors such as genetic, environmental exposure (black fly or Simulium nigrimanum or other insects, ultraviolet radiation), drug exposure (penicillin, cephalosporins, enalapril, rifampin, and nonsteroidal anti-inflammatory agents) as well as diet ( garlic, leek, onion, black pepper, red chili pepper, red wine, and tea that can induce pemphigus vulgaris and pemphigus foliaceus) are supported by some studies (Hertl & Sitaru, 2022).
Time Course
Duration: Pemphigus can be rapidly fatal to relatively benign (McCance & Huether, 2019)
Pattern or Prodrome of Symptoms: According to McCance & Huether (2019), pemphigus causes blisters on the skin. Signs and symptoms of the different types of the diseases are as follow:
Pemphigus vulgaris, the most common form , is characterized by oral lesions that precede onset of thin and fragile blisters on the skin of the face, scalp, and axilla. These blisters rupture easily and result in painful erosions.
Pemphigus vulgaris, a rare variant, is characterized by large blisters with tissue erosion and pustule that occur in tissue folds
Pemphigus foliaceus is mild form of pemphigus are constituted of blistering, erosions, scaling, crusting, and erythema that develop on the face and chest.
Pemphigus erythematosus is usually associated with systemic lupus erythematosus. They are diffused.
Paraneoplastic pemphigus, the most severe form, can be fatal. “This form of pemphigus is associated with lymphoproliferative neoplasms. Internal organs, including lungs, thyroid, kidney, smooth muscle, and the gastrointestinal tract, also are involved, leading to the term paraneoplastic autoimmune multiorgan syndrome. In contrast to pemphigus vulgaris, the lesions develop from inflammatory papules or macules rather than normal skin (p. 4847)
IgA pemphigus, the most begin form, can be found on the epidermis.
Pemphigus herpetiformis, a rare form, has the appearance of herpes lesions called dermatitis herpetiformis.
Clinical Presentation with Classic S&S
Key & Differentiating Features: Patients affected with pemphigus vulgaris develop mucosal lesions most commonly present in the oral cavity that is the initial site of the disorder. Other mucosal sites such as the conjunctiva, nose, esophagus, vulva, vagina, cervix, as well as the anus can also be affected. Erosions remain the clinical finding because of mucosal blisters abrade quickly. Oral erosions can be painful, with chewing and swallowing that can cause malnutrition and weigh loss. Cutaneous erosions are flaccid blisters with normal appearance or erythematous skin. They are painful, can rupture and bleed easily (Hertl & Sitaru, 2022).
Must Have Features: Flaccid, and mucosal cutaneous blisters/erosions. Painful erosions with the onset, in most cases, of lesions in the oral cavity. (Costan et al., 2021).
Rejecting Features: Other types of erosions or blisters that do not involve autoantibodies commonly found in diagnostic tests of pemphigus.
Mechanism of Disease Process
Pathophysiology: “Patient affected with pemphigus vulgaris, antibodies are oriented against desmosomal cadherins, Dsg1 and Dsg3, but other autoantibodies have been identified targeting other metabolic and structural proteins, such as Dsc1 and Dsc3 desmocolins, mitochondrial antigens, hSPCA1, thyroid peroxidase, muscarinic and nico? tinic acetylcholine receptors, plakoglobin, E?cadherin and plakophilin 3 (9,12?14)” (Costan et al, 2021, p. 2). Assessment is based on the clinical, histologic, immunopathologic, and serologic findings made through the assessment of clinical, histologic, immunopathologic, and serologic findings (Hertl & Sitaru, 2022).
Diagnostic Test(s) & Findings
Clinicians confirm pemphigus with diagnostic tests results that involve histologic, clinical, immunologic, and serologic findings. For example, laboratory test of hematoxylin and eosin (H&E) staining for biopsy of lesions on the skin or the mucus, direct immunofluorescence (DIF) for biopsy of perilesional skin, serum collection for enzyme-linked immunosorbent assay (ELISA), and indirect immunofluorescence (IIF).
References
Costan, V., Popa, C., Porumb-Andrese, E., & Toader, M. P. (2021). Comprehensive
review on the pathophysiology, clinical variants and management of
pemphigus (review). Experimental and Therapeutic Medicine, 22(5)
doi:https://doi.org/10.3892/etm.2021.10770
McCance, K. L., & Huether, S. E. (2019). Pathophysiology: The biologic basis for disease in
adults and children (8th ed.). Elsevier.
Hertl, M. Sitaru, C. (2022). Pathogenesis, clinical manifestations, and diagnosis of pemphigus.
UpToDate, Retrieved January 18, 2023, from: https://www-uptodate-
com/contents/pathogenesis-clinical-manifestations-and-diagnosis-of-pemphigus
