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Esen K. Akpek - One of the best experts on this subject based on the ideXlab platform.
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characterization of progressive Cicatrizing Conjunctivitis with negative immunofluorescence staining
American Journal of Ophthalmology, 2020Co-Authors: Jae Young You, Charles G Eberhart, Sezen Karakus, Esen K. AkpekAbstract:Purpose To characterize the clinical features of patients with direct immunofluorescence (DIF)-negative mucous membrane pemphigoid (MMP). Design Retrospective case series. Methods Thirty-six patients who underwent a conjunctival biopsy for suspected MMP were included. Demographic and clinical information was collected. Main outcome measures included visual acuity, Foster stages, presence of extraocular involvement, history of autoimmune disease, and durations of follow-up. Results Thirty-two patients had a negative DIF. Of those, 2 had a positive DIF on repeat biopsy. Eleven showed progression of conjunctival scarring during a median follow-up of 42 months (range, 8-100 months) and were diagnosed with biopsy-negative MMP. Another 11 patients with a median follow-up of 54 months (range, 15-138 months) were diagnosed with Cicatrizing Conjunctivitis of other causes. The median visual acuity of patients with biopsy-negative MMP at presentation was significantly lower compared to patients with Cicatrizing Conjunctivitis of other causes (20/400 vs 20/40, P = .02). Conjunctival scarring score at presentation in both biopsy-positive and biopsy-negative MMP groups was significantly higher compared to patients with Cicatrizing Conjunctivitis of other causes (median Foster stage, 3 vs 1, P = .009; and 3 vs 1, P = .01, respectively). Conclusions Patients with progressive Cicatrizing Conjunctivitis likely have MMP in the absence of alternate diagnoses. Our findings emphasize that suspicion for MMP must remain high for patients who have Foster stage 3 conjunctival scarring on presentation or worsening of scarring during follow-up, even in the setting of negative DIF.
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the usage of a conjunctival flap to improve retention of boston type 1 keratoprosthesis in severe ocular surface disease
Ocular Immunology and Inflammation, 2016Co-Authors: Allen O Eghrari, Sumayya Ahmad, Pradeep Y Ramulu, N T Iliff, Esen K. AkpekAbstract:AbstractPurpose: The Boston keratoprostheses type 1 devices (KPro) are utilized in cases unfavorable to penetrating keratoplasty. The prognosis remains guarded in cases of ocular surface disease due to risk of tissue necrosis. We describe a novel surgical approach using a conjunctival flap with a delayed opening to improve retention.Methods: In three patients with advanced Cicatrizing Conjunctivitis, a Type 1 keratoprosthesis was stabilized using a full tarsal conjunctival flap. Three months postoperatively, an opening was created in the flap overlying the optical portion of the device.Results: All patients had no device related complications over a mean follow-up period of 17.7 months (range 15-21 months) and vision remained excellent at better than 20/200 for all patients.Conclusions: Utilization of a tarsal flap either primarily as part of a two stage modified technique or secondarily in cases of tissue necrosis and impending device extrusion might maximize retention of the type 1 KPro.
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Ocular Rosacea: Patient Characteristics and Follow-up
Ophthalmology, 1997Co-Authors: Esen K. Akpek, A. Merchant, V. Pinar, Charles Stephen FosterAbstract:Abstract Purpose: The purpose of this report is to review the presenting symptoms and signs, treatment regimens used, complications encountered, and outcome in a cohort of patients with ocular rosacea. Methods: The medical records of 131 patients with a diagnosis of ocular rosacea were reviewed retrospectively. Data were entered in a tabulated form, and a descriptive analysis was performed. Results: The age range at presentation was between 23 and 85 years (mean, 56 years). Cutaneous manifestations of rosacea were present in 112 of the patients at their first visit. The most common presenting symptoms were foreign body sensation and burning, and the most common signs were telangiectasia and irregularity of lid margins, and meibomian gland dysfunction. Thirteen patients had decreased visual acuity at the time of presentation due to corneal complications. Six of these patients required penetrating keratoplasty during the course of their disease. Seven patients had severe Cicatrizing Conjunctivitis at the time of referral. One hundred thirteen patients were treated with oral tetracycline derivatives. Seven patients were left with visual acuity less than 20/400, and one patient underwent enucleation for corneal perforation and endophthalmitis. Conclusions: Ocular rosacea is a common disease involving the skin and the eyes. It is widely underdiagnosed by many ophthalmologists despite the blinding potential. Successful therapy requires a multidisciplinary approach.
Erik Letko - One of the best experts on this subject based on the ideXlab platform.
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chronic Cicatrizing Conjunctivitis in a patient with epidermolysis bullosa acquisita
Archives of Ophthalmology, 2006Co-Authors: Erik Letko, Razzaque A Ahmed, Kailash C. Bhol, Fahd Anzaar, Victor L Perez, Stephen C FosterAbstract:Objective To describe a nonconventional diagnostic technique used to diagnose a case of Cicatrizing Conjunctivitis associated with epidermolysis bullosa acquisita. Methods Direct immunofluorescence of a biopsy specimen of the patient's conjunctiva was performed using fluorescein-conjugated rabbit antihuman antibodies against IgA, IgG, and IgM; complement C3; and fibrinogen. Immunoblot assay using healthy human skin as substrate was performed to investigate for the presence of antibodies in the patient's serum. After the diagnosis of systemic autoimmune disease was established, intravenous immunoglobulin therapy was administered. Results Direct immunofluorescence of the conjunctiva revealed linear deposition of IgA and IgG, and C3 at the epithelial basement membrane zone. Immunoblot analysis demonstrated the presence of IgG antibodies in patient serum directed against a 290-kDa protein in human skin. A diagnosis of epidermolysis bullosa acquisita was established. All signs and symptoms improved dramatically 4 months after initiation of intravenous immunoglobulin therapy and remained stable during follow-up. Conclusions Epidermolysis bullosa acquisita can manifest in the eye as chronic Cicatrizing Conjunctivitis indistinguishable from ocular cicatricial pemphigoid. A nonconventional diagnostic tool (immunoblot assay) might be helpful in establishing the diagnosis of an underlying systemic autoimmune disease in patients with chronic Cicatrizing Conjunctivitis. Intravenous immunoglobulin therapy was effective against chronic Cicatrizing Conjunctivitis associated with epidermolysis bullosa acquisita.
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linear iga bullous disease limited to the eye a diagnostic dilemma response to intravenous immunoglobulin therapy
Ophthalmology, 2000Co-Authors: Kailash C. Bhol, Stephen C Foster, Erik Letko, Razzaque A AhmedAbstract:Abstract Purpose 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. Design 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.
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the role of antibody to human β4 integrin in conjunctival basement membrane separation possible in vitro model for ocular cicatricial pemphigoid
Investigative Ophthalmology & Visual Science, 1999Co-Authors: Robert K Y Chan, Kailash C. Bhol, Erik Letko, C S Foster, N Tesavibul, R K Simmons, A R AhmedAbstract:Purpose To demonstrate the specific binding of autoantibodies present in the sera of patients with ocular cicatricial pemphigoid (OCP) to human beta4 integrin present in the normal human conjunctiva (NHC) and to study the role of OCP autoantibodies and antibody to human beta4 integrin in the pathogenesis of subepithelial lesion formation in OCP. Methods Indirect immunofluorescence assay and in vitro organ culture method using NHC were used. Sera and IgG fractions from 10 patients with OCP; immunoaffinity-purified OCP autoantibody; antibodies to human beta4, beta1, alpha6, and alpha5 integrins; and sera from patients with pemphigus vulgaris, bullous pemphigoid (BP), and chronic atopic and chronic ocular rosacea Cicatrizing Conjunctivitis; and normal human serum (NHS) were used. Results Nine of 10 OCP sera or IgG fractions, immunoaffinity-purified OCP autoantibody, antibodies to human beta4 and alpha6 integrins, and sera from patients with BP showed homogenous, smooth linear binding along the basement membrane zone (BMZ) of the NHC. NHS, antibodies to other integrins, and sera from patients with chronic Cicatrizing Conjunctivitis from other causes showed no such binding. When NHC was first absorbed with OCP sera and then reacted with anti-beta4 antibodies or vice versa, the intensity of the BMZ binding was dramatically reduced or completely eliminated, indicating that there were autoantibodies in OCP sera specific for the beta4 integrin. BMZ separation developed 48 to 72 hours after addition of total OCP sera, IgG fractions from OCP sera, immunoaffinity-purified autoantibodies from sera of patients with OCP, or anti-beta4 antibodies to the NHC cultures, but not after addition of normal control sera, sera from patients with chronic Cicatrizing Conjunctivitis from causes other than OCP, or sera from patients with OCP in clinical remission. Conclusion Circulating anti-beta4 integrin antibody may have an important role in the pathogenesis of OCP.
Kailash C. Bhol - One of the best experts on this subject based on the ideXlab platform.
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chronic Cicatrizing Conjunctivitis in a patient with epidermolysis bullosa acquisita
Archives of Ophthalmology, 2006Co-Authors: Erik Letko, Razzaque A Ahmed, Kailash C. Bhol, Fahd Anzaar, Victor L Perez, Stephen C FosterAbstract:Objective To describe a nonconventional diagnostic technique used to diagnose a case of Cicatrizing Conjunctivitis associated with epidermolysis bullosa acquisita. Methods Direct immunofluorescence of a biopsy specimen of the patient's conjunctiva was performed using fluorescein-conjugated rabbit antihuman antibodies against IgA, IgG, and IgM; complement C3; and fibrinogen. Immunoblot assay using healthy human skin as substrate was performed to investigate for the presence of antibodies in the patient's serum. After the diagnosis of systemic autoimmune disease was established, intravenous immunoglobulin therapy was administered. Results Direct immunofluorescence of the conjunctiva revealed linear deposition of IgA and IgG, and C3 at the epithelial basement membrane zone. Immunoblot analysis demonstrated the presence of IgG antibodies in patient serum directed against a 290-kDa protein in human skin. A diagnosis of epidermolysis bullosa acquisita was established. All signs and symptoms improved dramatically 4 months after initiation of intravenous immunoglobulin therapy and remained stable during follow-up. Conclusions Epidermolysis bullosa acquisita can manifest in the eye as chronic Cicatrizing Conjunctivitis indistinguishable from ocular cicatricial pemphigoid. A nonconventional diagnostic tool (immunoblot assay) might be helpful in establishing the diagnosis of an underlying systemic autoimmune disease in patients with chronic Cicatrizing Conjunctivitis. Intravenous immunoglobulin therapy was effective against chronic Cicatrizing Conjunctivitis associated with epidermolysis bullosa acquisita.
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linear iga bullous disease limited to the eye a diagnostic dilemma response to intravenous immunoglobulin therapy
Ophthalmology, 2000Co-Authors: Kailash C. Bhol, Stephen C Foster, Erik Letko, Razzaque A AhmedAbstract:Abstract Purpose 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. Design 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.
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the role of antibody to human β4 integrin in conjunctival basement membrane separation possible in vitro model for ocular cicatricial pemphigoid
Investigative Ophthalmology & Visual Science, 1999Co-Authors: Robert K Y Chan, Kailash C. Bhol, Erik Letko, C S Foster, N Tesavibul, R K Simmons, A R AhmedAbstract:Purpose To demonstrate the specific binding of autoantibodies present in the sera of patients with ocular cicatricial pemphigoid (OCP) to human beta4 integrin present in the normal human conjunctiva (NHC) and to study the role of OCP autoantibodies and antibody to human beta4 integrin in the pathogenesis of subepithelial lesion formation in OCP. Methods Indirect immunofluorescence assay and in vitro organ culture method using NHC were used. Sera and IgG fractions from 10 patients with OCP; immunoaffinity-purified OCP autoantibody; antibodies to human beta4, beta1, alpha6, and alpha5 integrins; and sera from patients with pemphigus vulgaris, bullous pemphigoid (BP), and chronic atopic and chronic ocular rosacea Cicatrizing Conjunctivitis; and normal human serum (NHS) were used. Results Nine of 10 OCP sera or IgG fractions, immunoaffinity-purified OCP autoantibody, antibodies to human beta4 and alpha6 integrins, and sera from patients with BP showed homogenous, smooth linear binding along the basement membrane zone (BMZ) of the NHC. NHS, antibodies to other integrins, and sera from patients with chronic Cicatrizing Conjunctivitis from other causes showed no such binding. When NHC was first absorbed with OCP sera and then reacted with anti-beta4 antibodies or vice versa, the intensity of the BMZ binding was dramatically reduced or completely eliminated, indicating that there were autoantibodies in OCP sera specific for the beta4 integrin. BMZ separation developed 48 to 72 hours after addition of total OCP sera, IgG fractions from OCP sera, immunoaffinity-purified autoantibodies from sera of patients with OCP, or anti-beta4 antibodies to the NHC cultures, but not after addition of normal control sera, sera from patients with chronic Cicatrizing Conjunctivitis from causes other than OCP, or sera from patients with OCP in clinical remission. Conclusion Circulating anti-beta4 integrin antibody may have an important role in the pathogenesis of OCP.
Stephen C Foster - One of the best experts on this subject based on the ideXlab platform.
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chronic Cicatrizing Conjunctivitis in a patient with epidermolysis bullosa acquisita
Archives of Ophthalmology, 2006Co-Authors: Erik Letko, Razzaque A Ahmed, Kailash C. Bhol, Fahd Anzaar, Victor L Perez, Stephen C FosterAbstract:Objective To describe a nonconventional diagnostic technique used to diagnose a case of Cicatrizing Conjunctivitis associated with epidermolysis bullosa acquisita. Methods Direct immunofluorescence of a biopsy specimen of the patient's conjunctiva was performed using fluorescein-conjugated rabbit antihuman antibodies against IgA, IgG, and IgM; complement C3; and fibrinogen. Immunoblot assay using healthy human skin as substrate was performed to investigate for the presence of antibodies in the patient's serum. After the diagnosis of systemic autoimmune disease was established, intravenous immunoglobulin therapy was administered. Results Direct immunofluorescence of the conjunctiva revealed linear deposition of IgA and IgG, and C3 at the epithelial basement membrane zone. Immunoblot analysis demonstrated the presence of IgG antibodies in patient serum directed against a 290-kDa protein in human skin. A diagnosis of epidermolysis bullosa acquisita was established. All signs and symptoms improved dramatically 4 months after initiation of intravenous immunoglobulin therapy and remained stable during follow-up. Conclusions Epidermolysis bullosa acquisita can manifest in the eye as chronic Cicatrizing Conjunctivitis indistinguishable from ocular cicatricial pemphigoid. A nonconventional diagnostic tool (immunoblot assay) might be helpful in establishing the diagnosis of an underlying systemic autoimmune disease in patients with chronic Cicatrizing Conjunctivitis. Intravenous immunoglobulin therapy was effective against chronic Cicatrizing Conjunctivitis associated with epidermolysis bullosa acquisita.
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linear iga bullous disease limited to the eye a diagnostic dilemma response to intravenous immunoglobulin therapy
Ophthalmology, 2000Co-Authors: Kailash C. Bhol, Stephen C Foster, Erik Letko, Razzaque A AhmedAbstract:Abstract Purpose 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. Design 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.
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chronic keratoConjunctivitis associated with congenital dyskeratosis and erythrokeratodermia variablis two rare genodermatoses
Ophthalmology, 1998Co-Authors: Amyna Merchant, Tongzheng Zhao, Stephen C FosterAbstract:Abstract Objective The purpose of the study was to report the ocular manifestations and immunohistopathologic description of two rare forms of genodermatosis: congenital dyskeratosis (CD) and erythrokeratodermia variabilis (EKV). Design Case reports. Participants Two patients with CD and EKV presenting with chronic keratoConjunctivitis were studied. Intervention Clinical photography to show ocular manifestations and dermatologic findings was performed. Conjunctival biopsy was performed to determine the histopathology and immunohistopathology. Results In the patient with EKV, the eyelid skin was dry, erythematous, and thickened. The lid margins showed plugging of some of the Meibomian glands, madarosis, and occasional trichiasis. The epibulbar conjunctiva was injected and had a moderate papillary reaction. Both corneas had pronounced vascularization and stromal scarring. Conjunctival biopsy results disclosed a striking number of degranulating mast cells and moderate numbers of plasma cells. Immunohistopathology showed pronounced immunoglobulin G (IgG)-positive cells in the stroma and immunoglobulin A (IgA) positivity in the epithelium. There was scattered immunoglobulin M (IgM) and moderate C3 and C4 positivity in the stroma. In CD, the patient had keratinized lid margins, entropion formation, trichiasis, Cicatrizing Conjunctivitis, and symblephra formation. The corneal surface was keratinized, with deep and superficial vascularization. Conjunctival biopsy specimens showed pronounced epithelial keratinization and squamous metaplasia. Degranulating mast cells and eosinophils were prominent in the stroma. Immunohistopathology showed C4 and immunoglobulin D positivity on the keratinized epithelial surface with rare foci of immunoglobulin E-positive cells. Basal epithelial cells were positive for IgA and IgG, and a large number of IgA and IgG plasma cells were present in the substantia propria. Conclusion To the authors' knowledge, these case reports represent the first clinical description of the ocular manifestation of EKV and the first immunohistopathologic description of the affected conjunctiva in EKV and CD, both of which should be considered in the differential diagnosis of genodermatosis associated with chronic keratoConjunctivitis.
Razzaque A Ahmed - One of the best experts on this subject based on the ideXlab platform.
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chronic Cicatrizing Conjunctivitis in a patient with epidermolysis bullosa acquisita
Archives of Ophthalmology, 2006Co-Authors: Erik Letko, Razzaque A Ahmed, Kailash C. Bhol, Fahd Anzaar, Victor L Perez, Stephen C FosterAbstract:Objective To describe a nonconventional diagnostic technique used to diagnose a case of Cicatrizing Conjunctivitis associated with epidermolysis bullosa acquisita. Methods Direct immunofluorescence of a biopsy specimen of the patient's conjunctiva was performed using fluorescein-conjugated rabbit antihuman antibodies against IgA, IgG, and IgM; complement C3; and fibrinogen. Immunoblot assay using healthy human skin as substrate was performed to investigate for the presence of antibodies in the patient's serum. After the diagnosis of systemic autoimmune disease was established, intravenous immunoglobulin therapy was administered. Results Direct immunofluorescence of the conjunctiva revealed linear deposition of IgA and IgG, and C3 at the epithelial basement membrane zone. Immunoblot analysis demonstrated the presence of IgG antibodies in patient serum directed against a 290-kDa protein in human skin. A diagnosis of epidermolysis bullosa acquisita was established. All signs and symptoms improved dramatically 4 months after initiation of intravenous immunoglobulin therapy and remained stable during follow-up. Conclusions Epidermolysis bullosa acquisita can manifest in the eye as chronic Cicatrizing Conjunctivitis indistinguishable from ocular cicatricial pemphigoid. A nonconventional diagnostic tool (immunoblot assay) might be helpful in establishing the diagnosis of an underlying systemic autoimmune disease in patients with chronic Cicatrizing Conjunctivitis. Intravenous immunoglobulin therapy was effective against chronic Cicatrizing Conjunctivitis associated with epidermolysis bullosa acquisita.
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linear iga bullous disease limited to the eye a diagnostic dilemma response to intravenous immunoglobulin therapy
Ophthalmology, 2000Co-Authors: Kailash C. Bhol, Stephen C Foster, Erik Letko, Razzaque A AhmedAbstract:Abstract Purpose 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. Design 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.