C3d immunohistochemistry on formalin-fixed tissue is a valuable tool in the diagnosis of bullous pemphigoid of the skin.
BACKGROUND
Direct immunofluorescence (DIF) testing is an important procedure in the diagnosis of autoimmune bullous dermatoses. We investigated the expression of C3d in formalin-fixed, paraffin-embedded tissue of autoimmune bullous dermatoses.


METHODS
The immunohistochemical expression of C3d in bullous pemphigoid (BP) (n = 32), pemphigoid gestationis (PG) (n = 3), pemphigus (n = 14), dermatitis herpetiformis Duhring (DHD) (n = 10), linear immunoglobulin A (IgA) dermatosis (n = 4), mixed forms of BP and linear IgA dermatosis (n = 2), and 44 controls was analyzed on formalin-fixed tissue.


RESULTS
Thirty-one of 32 cases (97%) of BP and 3 out of 3 cases (100%) of PG showed a linear positivity of C3d along the basement membrane. Only 3 out of 14 (21%) cases of pemphigus showed an intraepidermal intercellular expression of C3d. The two mixed forms of linear IgA dermatosis and BP showed a linear positivity of C3d along the basement membrane. All cases of DHD, linear IgA dermatosis and all of the controls were negative for C3d.


CONCLUSIONS
C3d immunohistochemistry is a valuable tool in the diagnosis of BP and PG of the skin with a sensitivity of at least 97%. Mixed forms of linear IgA dermatosis, and BP, DHD and linear IgA dermatosis can only be identified by DIF. A positive result may prompt serologic confirmation of BP without further need for DIF.
Pfaltz
Katrin
K
Kempf & Pfaltz Histological Diagnostics, CH-8042 Zürich, Switzerland.
Mertz
Kirsten
K
Rose
Christian
C
Scheidegger
Paul
P
Pfaltz
Madeleine
M
Kempf
Werner
W
D015933:Complement C3d / Q000378:metabolism*; D005557:Formaldehyde; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods*; D010391:Pemphigoid, Bullous / Q000175:diagnosis* / Q000378:metabolism; D012867:Skin / Q000196:embryology / Q000378:metabolism*
D005557:Formaldehyde; D015933:Complement C3d