Circulating bullous pemphigoid autoantibodies in the setting of negative direct immunofluorescence findings for bullous pemphigoid: A single-center retrospective review

• Published in: Journal of the American Academy of Dermatology Vol. 81; no. 2; pp. 472 - 479
• Main Authors: Wang, Michael, Lehman, Julia S., Camilleri, Michael J., Drage, Lisa A., Wieland, Carilyn N.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-08-2019
• Subjects: Aged; Aged, 80 and over; Autoantibodies - blood; autoantibody; Autoantigens - immunology; basement membrane zone; bullous diseases; Collagen Type XVII; Dystonin - immunology; Enzyme-Linked Immunosorbent Assay; Female; Fluorescent Antibody Technique, Direct; Humans; immunobullous; immunofluorescence; Male; medical dermatology; Middle Aged; Non-Fibrillar Collagens - immunology; pemphigoid; Pemphigoid, Benign Mucous Membrane - blood; Pemphigoid, Benign Mucous Membrane - diagnosis; Pemphigoid, Benign Mucous Membrane - immunology; Pemphigoid, Bullous - blood; Pemphigoid, Bullous - diagnosis; Pemphigoid, Bullous - immunology; Predictive Value of Tests; Retrospective Studies; DIF; autoantibody; ICD-10; immunobullous; IIF; medical dermatology; IQR; BMZ; BP; bullous diseases; MMP; ANA; NPV; pemphigoid; PPV; basement membrane zone; immunofluorescence; ELISA
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1016/j.jaad.2019.03.062

Bullous pemphigoid (BP) autoantibody levels are generally elevated in patients with BP but can be present nonspecifically in patients without BP.
To analyze the clinical findings of patients with elevated BP180 or BP230 autoantibody levels and negative direct immunofluorescence (DIF) study findings.
We retrospectively reviewed records of patients seen at our institution during January 1, 2005-December 31, 2015, who were positive for BP180 or BP230 autoantibodies and had a negative DIF study finding. These patients' demographic characteristics and BP180 and BP230 levels were compared with those of a BP control group who were positive for BP180 or BP230 autoantibodies and had positive DIF study findings.
We identified 208 patients with BP autoantibodies but without positive DIF study findings. These patients' mean age and enzyme-linked immunosorbent assay values were significantly lower than those of the control group. Dermatitis was the most common final clinical diagnosis. Of the 208 patients, 41 (19.7%) had at least 2 years' follow-up. Four patients had positive DIF results upon repeating the test and ultimately received pemphigoid diagnoses.
Retrospective design with limited follow-up.
Patients might harbor serum BP autoantibodies in the context of a wide range of dermatoses. Low positive BP180 and BP230 autoantibody levels should not be overinterpreted as evidence for BP in the setting of a negative DIF.