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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 .
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 B‐cell proliferations and thymoma or thymoma‐like neoplasms; specifically Non Hodgkin's lymphoma (42%) Dermatitis herpetiformis: Lymphoma Porphyria cutanea tarda and variegate porphyrias Hepatocellular Carcinoma Erythroderma and exfoliative dermatitis Mycosis fungoides or Sézary syndrome Pyoderma gangrenosum
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.
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 ...
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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