Linear IgA bullous disease limited to the eye: a diagnostic dilemma: response to intravenous immunoglobulin therapy.
OBJECTIVE
To report on a diagnostic dilemma and treatment challenge in a patient with chronic cicatrizing conjunctivitis without involvement of skin and other mucous membranes persisting for 6 years and not responding to topical and systemic steroids.


METHODS
Interventional case report.


METHODS
We performed direct immunofluorescence of the conjunctiva with fluorescein-conjugated rabbit antihuman antibodies against immunoglobulin A, G, and M, complement 3 component, and fibrinogen. To investigate the presence of circulating antibodies in patient's serum, indirect immunofluorescence using normal human conjunctiva, normal human skin, and monkey esophagus as substrate was done. In addition, we did immunoblot analysis using normal human epidermis as substrate to determine the molecular weight of an antigen. The patient was treated with intravenous immunoglobulin (IVIg). The correlation between the titer of circulating antibodies and the activity of conjunctival inflammation at various intervals during the course of IVIg therapy was demonstrated by immunoblot assay with serial dilutions of the patient's serum. The highest dilution at which the binding was visible was considered the titer.


RESULTS
Direct immunofluorescence of the conjunctiva and indirect immunofluorescence with both salt split skin and conjunctiva as substrate disclosed linear deposition of immunoglobulin A (IgA) at the epithelial basement membrane. Immunoblot analysis demonstrated the presence of IgA circulating antibodies in patient's serum directed against a 97kDa protein in human epidermis. A continuous decrease in the titer of these antibodies correlating to improvement of clinical symptoms was observed during IVIg therapy.


CONCLUSIONS
Use of a nonconventional diagnostic tool (immunoblot analysis), in addition to conventional immunohistologic studies, might be helpful in establishing the diagnosis of patients with chronic cicatrizing conjunctivitis. On the basis of results of these laboratory tests and clinical presentation, we believe that this patient has linear IgA bullous disease limited to the eye. IVIg therapy decreased the titer of circulating antibodies and induced a remission in this patient.
The Massachusetts Eye and Ear Infirmary, Department of Ophthalmology, Uveitis and Immunology Service, Harvard Medical School, Boston, Massachusetts, USA.
E
E
Letko
K
K
Bhol
C S
CS
Foster
A R
AR
Ahmed
D000368:Aged; D001323:Autoantibodies / Q000032:analysis; D001324:Autoantigens / Q000276:immunology; D001485:Basement Membrane / Q000276:immunology / Q000473:pathology; D002908:Chronic Disease; D003228:Conjunctiva / Q000276:immunology / Q000473:pathology*; D003231:Conjunctivitis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D019085:Fluorescent Antibody Technique, Direct; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D015151:Immunoblotting; D007070:Immunoglobulin A / Q000276:immunology*; D007071:Immunoglobulin A, Secretory / Q000032:analysis; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D008565:Membrane Proteins / Q000276:immunology*; D010390:Pemphigoid, Benign Mucous Membrane / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D012867:Skin / Q000276:immunology
D001323:Autoantibodies; D001324:Autoantigens; D007070:Immunoglobulin A; D007071:Immunoglobulin A, Secretory; D016756:Immunoglobulins, Intravenous; D008565:Membrane Proteins