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Paraneoplastic pemphigus (PNP) is an autoimmune disorder stemming from an underlying tumor. It is hypothesized that antigens associated with the tumor trigger an immune response resulting in blistering of the skin and mucous membranes .
Symptomatic features of paraneoplastic syndrome cultivate in four ways: endocrine, neurological, mucocutaneous, and hematological.The most common presentation is a fever (release of endogenous pyrogens often related to lymphokines or tissue pyrogens), but the overall picture will often include several clinical cases observed which may specifically simulate more common benign conditions.
The prognosis with treatment is generally good and much better than in the classic paraneoplastic syndromes. In one of the two biggest case series, patients with anti-CASPR2 antibodies without tumours improved on average from a Modified Rankin Score (MRS) of 4 (moderate severe disability) to an MRS of 1 (no significant disability despite ...
5744 19227 Ensembl ENSG00000087494 ENSMUSG00000048776 UniProt P12272 P22858 RefSeq (mRNA) NM_002820 NM_198964 NM_198965 NM_198966 NM_008970 RefSeq (protein) NP_002811 NP_945315 NP_945316 NP_945317 n/a Location (UCSC) Chr 12: 27.96 – 27.97 Mb Chr 6: 147.15 – 147.17 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse Parathyroid hormone-related protein (PTHrP) is a proteinaceous ...
LEMS is often associated with lung cancer (50–70%), specifically small-cell carcinoma, [4] making LEMS a paraneoplastic syndrome. [5] Of the people with small-cell lung cancer, 1–3% have LEMS. [3] In most of these cases, LEMS is the first symptom of the lung cancer, and it is otherwise asymptomatic. [3]
Paraneoplastic syndrome, Guillain-Barré syndrome, diabetes, amyloidosis, Sjogren's syndrome, and Morvan syndrome. Autoimmune autonomic ganglionopathy is a type of immune-mediated autonomic failure that is associated with antibodies against the ganglionic nicotinic acetylcholine receptor present in sympathetic , parasympathetic , and enteric ...
Although many patients have an underlying cancer, the prognosis is determined by the severity of the neurological symptoms produced by the encephalitis. Compared to other paraneoplastic encephalitides, anti-Hu associated encephalitis has an especially poor prognosis.
POEMS syndrome (also termed osteosclerotic myeloma, Crow–Fukase syndrome, Takatsuki disease, or PEP syndrome) is a rare paraneoplastic syndrome caused by a clone of aberrant plasma cells. The name POEMS is an acronym for some of the disease's major signs and symptoms ( p olyneuropathy , o rganomegaly , e ndocrinopathy , m yeloma protein , and ...