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Test Catalog

Test ID: PNPAB    
Paraneoplastic Pemphigus Antibody (IgG), Serum

Useful For Suggests clinical disorders or settings where the test may be helpful

Diagnosis of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS) in the setting of erosive or lichenoid mucocutaneous disease

Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test

Paraneoplastic pemphigus (PNP; also paraneoplastic autoimmune multiorgan syndrome: PAMS, to denote the systemic nature of the syndrome) is an autoimmune mucocutaneous blistering disease affecting adults or rarely children that generally heralds the presence of an underlying malignancy.

 

PNP/PAMS can be defined and identified by a combination of the following features: 1) painful stomatitis and a polymorphous cutaneous eruption with lesions that may be blistering, lichenoid, erythema multiforme-like or morbilliform; 2) variable histopathologic findings, including acantholysis, lichenoid, or interface change; 3) variable direct immunofluorescence findings from a perilesional biopsy, often demonstrating deposition of IgG and complement in the epidermal intercellular spaces, granular/linear complement deposition along the epidermal basement membrane zone, and/or a lichenoid tissue reaction; 4) indirect immunofluorescence evidence of cell surface deposition on monkey esophagus and/or rat bladder epithelium, 5) ELISA evidence of serum autoantibodies against desmogleins 1 or 3, and possibly against bullous pemphigoid (BP) 180 and 230 antigens. The incidence of the disease is unknown but it is less common than pemphigus vulgaris (PV) or foliaceus (PF). Clinical features of the disease can mimic those seen in a drug reaction, erythema multiforme, Stevens-Johnson syndrome, pemphigus, lichen planus, or toxic epidermal necrolysis.

 

PNP/PAMS is associated in the majority of cases with non-Hodgkin lymphoma, chronic lymphocytic leukemia, thymoma, or Castleman disease. A serious complication includes bronchiolitis obliterans, which may lead to respiratory failure.

Reference Values Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.

Report as positive or negative.

Negative in normal individuals.

Interpretation Provides information to assist in interpretation of the test results

In the appropriate clinical setting, a positive result can support a diagnosis of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS). However, correlation with clinical features, histopathologic findings, results of serum studies (such as indirect immunofluorescence on monkey esophagus substrate and ELISA for Dsg1/3) is required for a final diagnosis. As the test is not entirely sensitive, a negative test result does not exclude the possibility of PNP/PAMS.

Cautions Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances

Test results must be interpreted in the patient's individual clinical context.

Clinical Reference Recommendations for in-depth reading of a clinical nature

1. Anhalt GJ, Kim SC, Stanley JR, et al: Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med 1990 Dec 20;323(25):1729-1735

2. Anhalt GJ, Aris-Abdo L, Bonitz P, Labib RS: Antigen specificity of paraneoplastic pemphigus: predictive value of diagnostic techniques based on the study of 17 patients and 135 control subjects. J Invest Dermatol 1992;98:580. Abstract

3. Liu AY, Valenzuela R, Helm TN, et al: Indirect immunofluorescence on rat bladder transitional epithelium: a test with high specificity for Paraneoplastic pemphigus. J Am Acad Dermatol 1993;28:696-699

4. Camisa C, Helm TN: Paraneoplastic pemphigus is a distinct neoplasia-induced autoimmune disease. Arch Dermatol 1993 Jul;129(7):883-886