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8041
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.
Useful For: Confirming a diagnosis of pemphigoid, pemphigus, epidermolysis bullosa acquisita, or
bullous lupus erythematosus Monitoring therapeutic response in patients with pemphigus
Interpretation: 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.
Reference Values:
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.
Clinical References: 1. Immunopathology of the Skin. Edited by EH Beutner, TP Chorzelski, V
Kumar. 3rd 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
Cutaneous Immunofluorescence, Biopsy
Clinical Information: Skin or mucosal tissue from patients with autoimmune bullous diseases,
connective tissue disease, vasculitis, lichen planus, and other inflammatory conditions often contains
bound immunoglobulin, complement, and/or fibrinogen. Biopsy specimens are examined for the presence
of bound IgG, IgM, IgA, third component of complement (C3), and fibrinogen. For your convenience, we
recommend utilizing cascade testing for celiac disease. Cascade testing ensures that testing proceeds in an
algorithmic fashion. The following cascades are available; select the appropriate 1 for your specific
patient situation. Algorithms for the cascade tests are available in Special Instructions. -CDCOM/89201
Celiac Disease Comprehensive Cascade: complete testing including HLA DQ -CDSP/89199 Celiac
Disease Serology Cascade: complete testing excluding HLA DQ -CDGF/89200 Celiac Disease
Comprehensive Cascade for Patients on a Gluten-Free Diet: for patients already adhering to a gluten-free
diet To order individual tests, see Celiac Disease Diagnostic Testing Algorithm in Special Instructions.
Useful For: Confirming a diagnosis of bullous pemphigoid, cicatricial pemphigoid, variants of
pemphigoid, all types of pemphigus, dermatitis herpetiformis, linear IgA bullous dermatosis, chronic
bullous disease of childhood, epidermolysis bullosa acquisita, porphyria cutanea tarda, bullous eruption of
lupus erythematosus, herpes gestationis, and atypical or mixed forms of bullous disease, systemic lupus
erythematosus, discoid lupus erythematosus, or other variants, vasculitis, lichen planus, and other
inflammatory diseases.
Interpretation: In pemphigus, direct immunofluorescence (IF) testing will show deposition of IgG, or
rarely IgA, and often complement C3 (C3) at the cell surface (intercelluar substances). In bullous
pemphigoid and cicatricial pemphigoid, direct IF study demonstrates deposition of IgG and C3 at the
basement membrane zone (BMZ) in a linear pattern. In cicatricial pemphigoid, a disease uncommonly
associated with circulating anti-BMZ antibodies, direct IF testing is particularly useful. Biopsy from
patients with dermatitis herpetiformis will show IgA concentrated in dermal papillae and/or in a granular
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