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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 .
Many forms of pemphigus can be identified in dogs using methods similar to those employed for humans. [14] Pemphigus vulgaris is rare in humans and animals, but is often fatal if left untreated. In dogs, the disease presents itself so similarly to the way it occurs in humans that dogs can be used as models for the disease in humans. [4]
Substrates used in indirect immunofluorescence studies for the different pemphigus variants Pemphigus variant Substrate Pemphigus foliaceous: Guinea pig esophagus Pemphigus vulgaris: Monkey esophagus IgA pemphigus: Cultured skin Paraneoplastic pemphigus: Rat bladder
Pemphigus foliaceus: IgG: Desmoglein 1: 160 Paraneoplastic pemphigus: IgG: Desmoglein 3 Desmoglein 1 BPAG1 [nb 1] Plectin Desmoplakin I [nb 2] Desmoplakin II Envoplakin Periplakin Alpha-2-macroglobulin-like-1: 130 160 230 500 250 210 210 190 170 Drug-induced pemphigus: IgG: Desmoglein 3 Desmoglein 1: 130 160 Subcorneal pustular dermatosis type ...
The least common and most severe type of pemphigus is paraneoplastic pemphigus (PNP). This disorder is a complication of cancer, usually lymphoma or Castleman's disease. It may precede the diagnosis of the tumor. Painful sores appear on the mouth, lips, and the esophagus.
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.
Paraneoplastic pemphigus: Non-Hodgkin lymphoma [nb 1] Chronic lymphocytic leukemia Castleman's disease [nb 1] Sarcoma Thymoma: Tripe palms: Lung cancer: Tripe palms with acanthosis nigricans: Stomach cancer: Pityriasis rotunda: Hepatocellular carcinoma Migratory thrombophlebitis Pancreatic adenocarcinoma Leser–Trélat sign: Gastrointestinal ...
If no lesions are present on examination it may be useful way of demonstrating reduced epithelial adhesion. In contrast, in Pemphigus, the epithelium tends to disintegrate rather than form a bulla. Nikolsky's sign is present in pemphigus and mucous membrane pemphigoid, but not in bullous pemphigoid.