-
International Journal of Molecular... Nov 2017Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Usually, oral, skin, and mucosal lesions are the earliest... (Review)
Review
Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by paraneoplastic pemphigus patients. The pathogenesis of paraneoplastic pemphigus is not yet completely understood, although some immunological aspects have been recently clarified. Because of its rarity, several diagnostic criteria have been proposed. Besides, several diagnostic procedures have been used for the diagnosis, including indirect immunofluorescence, direct immunofluorescence, and ELISA. We reviewed the most recent literature, searching on PubMed "paraneoplastic pemphigus". We included also papers in French, German, and Spanish. We found 613 papers for "paraneoplastic pemphigus". Among them, 169 were review papers. Because of its varying clinical features, paraneoplastic pemphigus still represents a challenge for clinicians. Furthermore, diagnosis and management of paraneoplastic pemphigus requires close collaboration between physicians, including dermatologist, oncologist, and otorhinolaryngologist.
Topics: Diagnosis, Differential; Humans; Immunologic Factors; Paraneoplastic Syndromes; Pemphigus
PubMed: 29186863
DOI: 10.3390/ijms18122532 -
Actas Dermo-sifiliograficas May 2015
Topics: Emergencies; Emergency Service, Hospital; Health Services Misuse; Humans; Paraneoplastic Syndromes; Soft Tissue Infections; Vasculitis
PubMed: 25534165
DOI: 10.1016/j.ad.2014.07.008 -
Actas Dermo-sifiliograficas Dec 2017Paraneoplastic pemphigus (PNP), a subset of pemphigus, is a unique autoimmune blistering condition that can affect multiple organs other than the skin. It is a... (Review)
Review
Paraneoplastic pemphigus (PNP), a subset of pemphigus, is a unique autoimmune blistering condition that can affect multiple organs other than the skin. It is a life-threatening disease associated with an underlying malignancy, most commonly of lymphoproliferative origin. The clinical picture may resemble pemphigus, pemphigoid, erythema multiforme, graft-versus-host disease, or lichen planus. The earliest and most consistent finding is a painful, severe, chronic and often recalcitrant stomatitis. Treatment of PNP is difficult. Immunosuppressive agents are required to decrease blistering, and treating the underlying tumor may control autoantibody production. In this review, we included essential diagnostic aspects of PNP and the most useful treatment options in the dermatologist practice.
Topics: Antigens, Neoplasm; Autoantibodies; Autoantigens; B-Lymphocytes; Bronchiolitis Obliterans; Castleman Disease; Diagnosis, Differential; Humans; Immunity, Cellular; Immunoglobulins, Intravenous; Immunosuppressive Agents; Paraneoplastic Syndromes; Pemphigus; Prognosis; Rituximab; Skin Diseases, Vesiculobullous; Stomatitis
PubMed: 28801011
DOI: 10.1016/j.ad.2017.04.024 -
BMJ Case Reports Jan 2015
Topics: Aged, 80 and over; Carcinoma; Female; Humans; Pancreatic Neoplasms; Panniculitis; Paraneoplastic Syndromes
PubMed: 25631757
DOI: 10.1136/bcr-2014-206980 -
Problemy Endokrinologii Jul 2022Tumor induced osteomalacia is a rare acquired disease. The cause is a mesenchymal tumor secreting fibroblast growth factor 23 (FGF23). An excessive amount of FGF 23...
Tumor induced osteomalacia is a rare acquired disease. The cause is a mesenchymal tumor secreting fibroblast growth factor 23 (FGF23). An excessive amount of FGF 23 disrupts the metabolism of phosphorus and vitamin D, which leads to severe paraneoplastic syndrome, manifested in the form of multiple fractures, severe pain in the bones and generalized myopathy. With oncogenic osteomalacia, a complete cure is possible with radical resection of the tumor. Unfortunately, localization, small size of formations and rare frequency of occurrence lead to the fact that the disease remains unrecognized for a long time and leads to severe, disabling consequences. A step-by-step approach to diagnosis improves treatment outcomes. First, a thorough anamnesis is collected, then functional visualization is performed and the diagnosis is confirmed by anatomical visualization of the tumor. After that, the method of choice is a surgical treatment. If resection is not possible, then conservative therapy with active metabolites of vitamin D and phosphorus salts is indicated. New therapeutic approaches, such as the antibody to FGF23 or the pan-inhibitor of receptors to FGF, are actively developing. This article provides an overview of modern approaches to the diagnosis and treatment of this disease.
Topics: Humans; Osteomalacia; Neoplasms, Connective Tissue; Paraneoplastic Syndromes; Fibroblast Growth Factors; Vitamin D; Phosphorus
PubMed: 36337019
DOI: 10.14341/probl13130 -
Magyar Onkologia Jun 2023The term paraneoplastic syndrome refers to the conditions when tumor-related symptoms are not caused by the size, invasion or metastasis of a tumor, but due to soluble...
The term paraneoplastic syndrome refers to the conditions when tumor-related symptoms are not caused by the size, invasion or metastasis of a tumor, but due to soluble mediators produced or an immune reaction induced by a tumor. Paraneoplastic syndromes occur in about 8% of all malignant tumors. Hormone-related paraneoplastic syndromes are termed paraneoplastic endocrine syndromes. In this short synopsis, the main clinical and laboratory characteristics of the most important paraneoplastic endocrine syndromes are presented including humoral hypercalcemia, the syndrome of inappropriate ADH secretion, ectopic ACTH syndrome. Two very rare diseases, paraneoplastic hypoglycemia and tumor-induced osteomalatia are also briefly presented.
Topics: Humans; Paraneoplastic Endocrine Syndromes; Paraneoplastic Syndromes; Autoantibodies; Rare Diseases
PubMed: 37314070
DOI: No ID Found -
Indian Journal of Ophthalmology Dec 2019
Topics: Antimetabolites, Antineoplastic; Female; Fluorescein Angiography; Humans; Intraocular Lymphoma; Intravitreal Injections; Lymphoma, Large B-Cell, Diffuse; Methotrexate; Middle Aged; Paraneoplastic Syndromes, Ocular; Retinal Neoplasms; Tomography, Optical Coherence; Visual Acuity
PubMed: 31755451
DOI: 10.4103/ijo.IJO_1697_19 -
Neurology Nov 2018To determine the proportion of true and false positives from paraneoplastic panels and effects on downstream testing/treatment.
OBJECTIVE
To determine the proportion of true and false positives from paraneoplastic panels and effects on downstream testing/treatment.
METHODS
Using a retrospective cohort study design, we identified 500 consecutive patients with Mayo paraneoplastic autoantibody testing and performed chart abstraction. Paraneoplastic presentation types were categorized into probable, possible, and other by consensus. True positives were defined as a positive antibody titer with no other explanation found in addition to one of the following: syndrome known to be associated with the antibody, clinical improvement with treatment, and new malignancy. Comparisons of diagnostic testing and treatments between false and true positives were performed. Multivariable logistic regression was used to evaluate associations between patient-level factors and true positives.
RESULTS
The mean (SD) age of the population was 55.4 (17.1) years, and 55.4% were female, with 1.3 (1.2) years of follow-up. Of the 500 tests, 87 (17.4%, 95% confidence interval [CI] 14.1%-20.7%) were positive and 62 (71.3%, 95% CI 61.8%-80.8%) of these were false positives. Of those with a possible/other presentation (n = 369), 2 (0.5%, 95% CI 0.0%-1.0%) were true positives. CT of the chest (30.7% vs 11.8%, ≤ 0.01) was performed more often in false positives than true negatives. Probable presentation type (odds ratio [OR] 57.9, 95% CI 12.5-268.0) and outpatient setting (OR 8.7, 95% CI 2.4-31.8) were associated with true-positive results.
CONCLUSION
Paraneoplastic tests result in a large proportion of false positives, particularly in those with clinical presentations that are not well established as paraneoplastic diseases. Future work should construct panels targeted to specific clinical presentations and ensure that tests are ordered in the appropriate clinical context.
Topics: Adult; Aged; Autoantibodies; False Positive Reactions; Female; Humans; Male; Middle Aged; Paraneoplastic Syndromes
PubMed: 30366974
DOI: 10.1212/WNL.0000000000006577 -
CMAJ : Canadian Medical Association... Dec 2018
Topics: Adenocarcinoma; Aged; Dermatitis, Exfoliative; Dermatomyositis; Facial Dermatoses; Female; Hand Dermatoses; Humans; Paraneoplastic Syndromes; Stomach Neoplasms
PubMed: 30530613
DOI: 10.1503/cmaj.180868 -
Medicina 2016
Topics: Aged; Female; Humans; Hypoglycemia; Liver Neoplasms; Paraneoplastic Syndromes; Pleural Neoplasms; Solitary Fibrous Tumors; Syndrome; Tomography, X-Ray Computed
PubMed: 27723620
DOI: No ID Found