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Sorted By Test Name - Mayo Medical Laboratories

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BP<br />

88874<br />

1 0.35-0.69 Equivocal<br />

2 0.70-3.49 Positive<br />

3 3.50-17.4 Positive<br />

4 17.5-49.9 Strongly positive<br />

5 50.0-99.9 Strongly positive<br />

6 > or =100 Strongly positive Reference values<br />

apply to all ages.<br />

Clinical References: Homburger HA: Allergic diseases. In Clinical Diagnosis and Management by<br />

Laboratory Methods. 21st edition. Edited by McPherson RA, Pincus MR. WB Saunders, Publ, New York,<br />

Chapter 53, Part VI, pp. 961-971, 2007<br />

Bullous Pemphigoid, BP180 and BP230, IgG Antibodies, Serum<br />

Clinical Information: Bullous pemphigoid (BP) is chronic pruritic blistering disorder found mainly<br />

in aged persons, characterized by the development of tense blisters over an erythematous or urticarial<br />

base. IgG antibasement membrane zone antibodies are found in the serum of patients, and linear IgG and<br />

C3 sediment is found on the basement membrane zone of the lesion. Several well characterized variants<br />

exist including localized, mucous membrane predominant and pemphigoid gestationis, also referred to as<br />

herpes gestationis. Target antigens of the autoantibodies in BP patient serum are BP230 and BP180 also<br />

called BPAG1 and BPAG2. Molecular weight of these antigens is 230 kD and 180 kD, respectively.<br />

BP180 is thought to be the direct target of the autoantibody because of its location along the basement<br />

membranes, and the autoantibody against BP230 is thought to be secondarily produced.<br />

Useful For: Bullous pemphigoid (BP) BP180 and BP230 enzyme-linked immunosorbent assay are<br />

sensitive, objective, and specific tests that should be considered as an initial screening test in the diagnosis<br />

of pemphigoid and its variants. To compare these results with the standard serum test of indirect<br />

immunofluorescence utilizing monkey esophagus substrate.<br />

Interpretation: Antibodies to bullous pemphigoid (BP) BP180 and BP230 have been shown to be<br />

present in most patients with pemphigoid. Adequate sensitivities and specificity for disease are<br />

documented and <strong>Mayo</strong>â€s experience demonstrates a very good correlation between BP180 and BP230<br />

results and the presence of pemphigoid (see “Supportive Dataâ€). However, in those patients strongly<br />

suspected to have pemphigoid, either by clinical findings or by routine biopsy, and in whom the<br />

BP180/BP230 assay is negative, follow-up testing by #8052 “Cutaneous Immunofluorescence<br />

Antibodies (IgG), Serum" is recommended. Antibody titer correlates with disease activity in many<br />

patients. Patients with severe disease can usually be expected to have high titers of antibodies to BP.<br />

Titers are expected to decrease with clinical improvement.<br />

Reference Values:<br />

BP180<br />

or =9.0 U (positive)<br />

BP230<br />

or =9.0 U (positive)<br />

Clinical References: 1. Liu Z, Diaz LA, Troy JL: A passive transfer model of the organ-specific<br />

autoimmune disease, bullous pemphigoid, using antibodies generated against the hemidesmosomal<br />

antigen, BP180. J Clin Invest 1993;92:2480-2488 2. Matsumura K, Amagai M, Nishikawa T, Hashimoto<br />

T: The majority of bullous pemphigoid and herpes gestationes serum samples react with the NC16a<br />

domain of the e180-kD bullous pemphigoid antigen. Arch Dematol Res 1996;288:507-509 3. Stanley JR,<br />

Hawley-Nelson P, Yuspa SH, et al: Characterization of bullous pemphigoid antigen: a unique basement<br />

Current as of January 4, 2013 7:15 pm CST 800-533-1710 or 507-266-5700 or <strong>Mayo</strong><strong>Medical</strong><strong>Laboratories</strong>.com Page 309

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