|Values are valid only on day of printing.|
Confirming a diagnosis of pemphigoid, pemphigus, epidermolysis bullosa acquisita, or bullous lupus erythematosus
Monitoring therapeutic response in patients with pemphigus
IgG anti-basement zone (BMZ) antibodies are produced by patients with pemphigoid. In most patients with bullous pemphigoid, serum contains IgG anti-BMZ antibodies, while in cicatricial pemphigoid circulating IgG anti-BMZ antibodies are found in a minority of cases. Sensitivity of detection of anti-BMZ antibodies is increased when serum is tested using sodium chloride (NaCl)-split human skin as substrate.
Circulating IgG anti-BMZ antibodies are also detected in patients with epidermolysis bullosa acquisita (EBA) and bullous eruption of lupus erythematosus.
IgG anti-cell surface (CS) antibodies are produced by patients with pemphigus. The titer of anti-CS antibodies generally correlates with disease activity of pemphigus.
See Method Description for special information pertaining to Herpes gestationis (pemphigoid) and paraneoplastic pemphigus.
Report includes presence and titer of circulating antibodies. If serum contains BMZ antibodies on split-skin substrate, patterns will be reported as: 1) epidermal pattern, consistent with pemphigoid or 2) dermal pattern, consistent with epidermolysis bullosa acquisita.
Negative in normal individuals. See Results of IF Testing* in Cutaneous Immunofluorescence Testing in Special Instructions.
Indirect immunofluorescence (IF) testing may be diagnostic when histologic or direct IF studies are only suggestive, nonspecific, or negative.
Anti-cell surface (CS) antibodies correlate with a diagnosis of pemphigus.
Anti-basement zone (BMZ) antibodies correlate with a diagnosis of bullous pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita (EBA), or bullous eruption of lupus erythematosus (LE).
If serum contains anti-BMZ antibodies, the pattern of fluorescence on sodium chloride(NaCl)-split skin substrate helps distinguish pemphigoid from EBA and bullous LE. Staining of the roof (epidermal side) or both epidermal and dermal sides of NaCl-split skin correlates with the diagnosis of pemphigoid, while fluorescence localized only to the dermal side of the split-skin substrate correlates with either EBA or bullous LE.
Results should be interpreted in conjunction with clinical information, histologic pattern, and results of direct immunofluorescence (IF) study. In particular, the finding of low titer (1:20 or 1:40) anti-CS antibodies should not be used alone (ie, without histologic or direct IF support) to confirm a diagnosis of pemphigus.
1. Immunopathology of the Skin. Edited by EH Beutner, TP Chorzelski, V Kumar. Third edition. New York, Wiley Medical Publication, 1987
2. Gammon WR, Briggaman RA, Inman AO 3rd, et al: Differentiating anti-lamina lucida and anti-sublamina densa anti-BMZ antibodies by indirect immunofluorescence on 1.0 M sodium chloride-separated skin. J Invest Dermatol 1984;82:139-144