Bullous Pemphigoid BP180 and BP230 Antibodies

Bullous pemphigoid is an autoimmune chronic pruritic blistering skin disease. Patients develop large, tense blisters over any erythematous base. Ruptured bullae may lead to erosions that are susceptible to bacterial infection.

Diagnosis depends on a combination of skin biopsy and serological studies. Histopathologic examination of a cutaneous biopsy of bullous pemphigoid shows subepidermal blistering with an inflammatory infiltrate consisting of eosinophils and neutrophils. 

Direct immunofluorescence on perilesional biopsy demonstrates a linear deposition of IgG and C3 along the basement membrane. Indirect immunofluorescence on human skin or monkey esophagus substrates displays serum antibody deposition in a linear fashion along the basement membrane.

Target antigens of the IgG autoantibodies in bullous pemphigoid are BP230 and BP180. 

These findings are the result of IgG autoantibodies targeting the hemidesmosomal proteins BP230 and BP180. They serve an important role in anchoring epidermal basal cells to the basement membrane.

Originally, serum antibodies were detected by indirect immunofluorescence using human skin or monkey esophagus. The identification of the target antigens allowed for the development of highly sensitive and specific enzyme-linked immunosorbent assays.

ELISA confirms that the circulating autoantibodies have specificity for BP180 and BP230. ELISA can also be used to monitor disease activity through the measurement of antibody titers.

BP180 and BP230 antibodies are detected using EuroImmun ELISA. Reference range for BP180 and BP230 antibodies is 0-20 RU/mL.

References

Nousari HC, Anhalt GJ. Pemphigus and bullous pemphigoid. Lancet. 1999;354(9179):667-672.

Kobayashi M, Amagai M, Kuroda-Kinoshita K, et al. BP180 ELISA using bacterial recombinant NC16a protein as a diagnostic and monitoring tool for bullous pemphigoid. J Dermatol Sci. 2002;30(3):224-232.

Mihai S, Sitaru C. Immunopathology and molecular diagnosis of autoimmune bullous diseases. J Cell Mol Med. 2007;11(3):462-481.