Paraneoplastic pemphigus (Clinical essentials): Dr. Aashritha yerneni скачать в хорошем качестве

Paraneoplastic pemphigus (Clinical essentials): Dr. Aashritha yerneni

📌𝐅𝐨𝐥𝐥𝐨𝐰 𝐨𝐧 𝐈𝐧𝐬𝐭𝐚𝐠𝐫𝐚𝐦:-   / drgbhanuprakash   📌𝗝𝗼𝗶𝗻 𝗢𝘂𝗿 𝗧𝗲𝗹𝗲𝗴𝗿𝗮𝗺 𝗖𝗵𝗮𝗻𝗻𝗲𝗹 𝗛𝗲𝗿𝗲:- https://t.me/bhanuprakashdr 📌𝗦𝘂𝗯𝘀𝗰𝗿𝗶𝗯𝗲 𝗧𝗼 𝗠𝘆 𝗠𝗮𝗶𝗹𝗶𝗻𝗴 𝗟𝗶𝘀𝘁:- https://linktr.ee/DrGBhanuprakash Paraneoplastic pemphigus (Clinical essentials): Dr. Aashritha yerneni Paraneoplastic pemphigus (PNP), also referred to as paraneoplastic autoimmune multiorgan syndrome (PAMS; some prefer this designation), is a variable multiorgan autoimmune syndrome with severe mucocutaneous disease that typically develops in the setting of current or past history of a lymphoreticular neoplasm, mainly B-cell or thymoma-like neoplasms. It is a rare disease, accounting for 3%-5% of all pemphigus cases. Most patients (approximately two-thirds) who develop PNP have a preceding history of the neoplasm. In the remaining patients, the onset of PNP will be the first clue that a lymphoreticular neoplasm is present. Specific neoplasms associated with PNP are Hodgkin disease (42%), chronic lymphocytic leukemia (29%), Castleman tumor (angiofollicular lymph node hyperplasia) (10%), thymoma (6%), spindle cell neoplasms (6%), and Waldenström macroglobulinemia (6%). The disease is thought to be due to the neoplastic process inducing both a humoral and cell-mediated humoral response. In particular, antibodies against tumor antigens cross-react with epithelial antigens, which is thought to cause the skin lesions. Immunoglobulin G (IgG) antibodies develop against multiple antigens, such as members of the plakin and desmoglein families. The disease is most commonly identified in an adult population aged 45-70 years, as this is the demographic group most likely to develop lymphoma. Patients with Castleman tumor tend to be younger, in the second or third decade of life. There is no association with sex, ethnicity, or geographic region, although there may be an association with DRB1*03 and HLA-Cw*14 alleles. Symptoms and signs include painful cutaneous and oral lesions secondary to the vesicles and bullae that form and subsequently rupture. Severe eye irritation may also be seen with conjunctival involvement, and esophageal, nasopharyngeal, vaginal, and penile mucosal lesions may also be seen. Pulmonary involvement, which is frequent, takes the form of bronchiolitis obliterans leading to dyspnea. Prognosis depends on the associated malignancy. Removal of some tumors (thymoma or Castleman disease) may induce disease remission. However, patients with other malignancies may deteriorate, with death due to sepsis, gastrointestinal bleeding, or organ failure. Decreased survival has also been noted in patients with a bullous pemphigoid-like and a toxic epidermal necrolysis (TEN)-like picture as well as the presence of bronchiolitis obliterans. #Paraneoplasticpemphigus #clinicalessentials #pemphigus #clinicalskills #usmlestep2cs #usmlestep2ck #usmle #clinicalvideos #clinicalprocedures