Epidermolysis bullosa acquisita diagnosed by direct immunoelectron microscopy of the conjunctiva.
OBJECTIVE
To describe for the first time the direct immunoelectron microscopic pattern of immune deposits on the conjunctival basement membrane in epidermolysis bullosa acquisita (EBA).


METHODS
Case reports.


METHODS
Two patients.


METHODS
Epidermolysis bullosa acquisita associated with cicatrizing conjunctivitis.


METHODS
Direct immunofluorescence and direct immunoelectron microscopy without freezing on conjunctival and skin biopsy specimens, indirect immunofluorescence, Western immunoblot analysis.


RESULTS
Results of direct immunoelectron microscopic examination of the conjunctiva showed the presence of immune deposits in the anchoring fibril zone, just beneath the lamina densa, in both patients. This finding was the same as the direct immunoelectron microscopic pattern shown in the skin of these patients, which is known to be very specific for EBA. Direct immunofluorescence was positive in the conjunctiva of only one patient. Indirect immunofluorescence and Western immunoblot analysis failed to detect circulating autoantibodies.


CONCLUSIONS
Direct immunoelectron microscopy on the conjunctiva is a useful diagnostic tool to differentiate EBA from other related autoimmune mucocutaneous blistering diseases.
Hoang-Xuan
T
T
Department of Ophthalmology, Hospital Bichat, Paris, France.
Robin
H
H
Heller
M
M
Caux
F
F
Prost
C
C
D000328:Adult; D000936:Antigen-Antibody Complex / Q000648:ultrastructure*; D001485:Basement Membrane / Q000648:ultrastructure; D015153:Blotting, Western; D003176:Complement C3 / Q000276:immunology; D003228:Conjunctiva / Q000648:ultrastructure*; D003231:Conjunctivitis / Q000175:diagnosis*; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis*; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000276:immunology; D008297:Male; D016253:Microscopy, Immunoelectron / Q000379:methods; D008875:Middle Aged; D011347:Procollagen / Q000276:immunology
D000936:Antigen-Antibody Complex; D003176:Complement C3; D007143:Immunoglobulin Heavy Chains; D011347:Procollagen