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1/10. Linear IgA dermatosis mimicking ocular cicatricial pemphigoid.

    PURPOSE: To describe an unusual case of cicatrizing conjunctivitis. methods AND RESULTS: A 59 year old male presented in July 1997 with a complaint of intermittent redness and discharge of the right eye. Medical history was significant for a buccal mucosal lesion biopsy taken two years earlier that revealed subepidermal fibrosis. Conjunctival biopsy, performed to rule out ocular cicatricial pemphigoid, yielded a diagnosis of linear IgA dermatosis, an unusual diagnosis not often considered by ophthalmologists. CONCLUSIONS: The diagnosis of cicatrizing conjunctivitis is often overlooked. Its importance as it pertains to the life threatening disease of ocular cicatricial pemphigoid is clear.
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keywords = conjunctivitis
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2/10. Cicatrizing conjunctivitis associated with paraneoplastic lichen planus.

    PURPOSE: To report two cases of cicatrizing conjunctivitis associated with paraneoplastic lichen planus. methods: case reports. RESULTS: Two patients were examined because of redness and discomfort in both eyes. A 63-year-old woman with follicular, small-cleaved cell lymphoma had cicatrizing conjunctivitis, stomatitis, vulvitis, and skin lesions. A 25-year-old man with malignant thymoma had cicatrizing conjunctivitis, erosive stomatitis, and penile papules. Histopathologic studies of conjunctiva and skin biopsy specimens in the first patient and labial biopsy specimens in the second revealed lichen planus. CONCLUSION: Paraneoplastic lichen planus is a possible cause of cicatrizing conjunctivitis associated with inflammatory skin and mucous membrane disease.
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ranking = 4
keywords = conjunctivitis
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3/10. Linear IgA bullous disease limited to the eye: a diagnostic dilemma: response to intravenous immunoglobulin therapy.

    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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ranking = 1
keywords = conjunctivitis
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4/10. Chronic cicatrizing conjunctivitis in a patient with ocular cicatricial pemphigoid and fatal wegener granulomatosis.

    PURPOSE: To describe a case of chronic cicatrizing conjunctivitis in a patient with ocular cicatricial pemphigoid and wegener granulomatosis. methods: Observational case report. A retrospective study. RESULTS: An 80-year-old man presented with chronic cicatrizing conjunctivitis, peripheral corneal thinning, and wegener granulomatosis, which were diagnosed by his referring physician based on clinical (recurrent epistaxis, sinus congestion) and histopathologic features of nasal mucosa (granulomatous inflammation, vasculitis). A conjunctival biopsy performed by us disclosed features of active wegener granulomatosis and ocular cicatricial pemphigoid, which indicate lack of control of both diseases with methotrexate treatment. The patient died of pulmonary complications from Wegener granulomatosis 1 week after our evaluation. CONCLUSION: Ocular cicatricial pemphigoid and wegener granulomatosis are both potentially fatal autoimmune diseases. Ocular involvement in wegener granulomatosis indicates poor control of the underlying systemic condition and is a marker for active vasculitis, which indicates the need for treatment with cyclophosphamide.
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ranking = 3
keywords = conjunctivitis
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5/10. Cicatricial pemphigoid with circulating IgA and IgG autoantibodies to the central portion of the BP180 ectodomain: beneficial effect of adjuvant therapy with high-dose intravenous immunoglobulin.

    Cicatricial pemphigoid (CP) is an autoimmune subepidermal blistering disease characterized by deposits of IgG, IgA, or C3 at the cutaneous basement membrane zone. CP may present with considerable variation regarding age, morphology of lesions, and mucosal involvement, which may heal with or without scarring. We describe a patient with CP who presented with circulating IgA and IgG autoantibodies to the epidermal side of salt-split human skin. By immunoblot analysis, the patient's IgA reacted with the soluble ectodomain of BP180 (LAD-1). This reactivity was mainly directed to the central portion of the BP180 ectodomain, a site that, to date, has not been described as the target of IgA autoantibodies. Different immunosuppressive treatment regimens including steroids and mycophenolate mofetil did not control this patient's disease, and severe scarring of the conjunctivae occurred with impairment of vision. Addition of adjuvant intravenous immunoglobulin (1 g/kg body weight on 2 consecutive days) every 4 weeks led to a dramatic improvement of conjunctivitis and gingivitis. Clinical improvement correlated with the serum's IgA immunoblot reactivity against LAD-1. Further studies on a larger number of patients with CP should try to correlate the specificity of autoantibodies in CP with the response to certain therapeutic regimens.
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keywords = conjunctivitis
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6/10. Bronchogenic squamous cell carcinoma presenting as cicatrizing conjunctivitis.

    PURPOSE: To report a case of cicatrizing conjunctivitis as the initial manifestation of a bronchogenic squamous cell carcinoma. methods: Case report of a 57-year-old patient with bilateral history of ocular pain, redness, and discharge, with no apparent predisposing factors. Examination revealed chemosis, pseudomembrane, and symblepharon formation, and multiple ulcerated pustular and vesicular periocular and perioral lesions literature review. RESULTS: skin biopsy was compatible with bullous pemphigoid. Two months later he developed respiratory symptoms, and a squamous cell carcinoma was diagnosed. CONCLUSIONS: This case demonstrates cicatrizing conjunctivitis as a possible paraneoplastic syndrome associated with squamous cell lung carcinoma.
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ranking = 3
keywords = conjunctivitis
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7/10. Childhood cicatricial pemphigoid with linear IgA deposits: a case report.

    A 16-year-old boy with a bullous eruption from the age of thirteen, also presented symptoms of scarring conjunctivitis and involvement of oral mucosal membranes. Linear IgA deposits were demonstrated in the basement membrane zone in skin and conjunctiva. The eruptions improved during therapy with aldesulfonsodium, but complete remission was not obtained. Gluten-free diet did not significantly influence the activity of the disease. The described patient seems to fit in a recently described entity of chronic bullous diseases.
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ranking = 0.5
keywords = conjunctivitis
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8/10. Topical tretinoin treatment for severe dry-eye disorders.

    Despite the diverse causes of dry-eye disorders, the ocular surface epithelia in these diseases all undergo squamous metaplasia, manifested by loss of goblet cells, mucin deficiency, and keratinization. These changes account for tearfilm instability, which leads to various ocular symptoms and corneal complications. This article reviews research in the use of topical tretinoin to treat severe dry-eye disorders. To classify squamous metaplasia into stages, a modified impression cytology technique was used to monitor the therapeutic effect of topical tretinoin ointment (0.01% or 0.1%, w/w) in 22 patients. This population had severe dry-eye disorders, including keratoconjunctivitis sicca, stevens-johnson syndrome, inactive ocular pemphigoid, drug-induced pseudopemphigoid, and surgery- or radiation-induced dry eyes. After treatment, clinical improvements were correlated with the reversal of squamous metaplasia as evidenced by the impression cytology technique. tretinoin may also be effective in treating conjunctival keratinization without dry eyes, as illustrated by a case study. This may represent the first reported attempt to treat ocular surface disorders by reversing diseased epithelium.
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ranking = 0.5
keywords = conjunctivitis
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9/10. Topical retinoid treatment for various dry-eye disorders.

    We evaluated the clinical efficacy of treating various dry-eye disorders using 0.01% and 0.1% (weight/weight) topical all-trans retinoic acid ointment. Twenty-two patients were selected and classified into four major groups: keratoconjunctivitis sicca (6 patients; 11 eyes), stevens-johnson syndrome (9 patients; 17 eyes), ocular pemphigoid or drug-induced pseudopemphigoid (3 patients; 6 eyes), and surgery or radiation-induced dry eye (4 patients; 4 eyes), based on the criterion that they remained symptomatic even under maximum tolerable conventional medical and/or surgical therapies. The results indicated that squamous metaplasia with mucin deficiency secondary to goblet cell loss and keratinization may be the basis for the development of clinical symptoms and morbidities, as these epithelial abnormalities were invariably present before treatment. After treatment, all patients demonstrated clinical improvements in symptoms, visual acuity, rose bengal staining, or Schirmer test. Most importantly, this topical vitamin a treatment caused the reversal of squamous metaplasia as evidenced by impression cytology. Therefore, this treatment may represent the first nonsurgical attempt to treat these disorders by reversing diseased ocular surface epithelium.
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ranking = 0.5
keywords = conjunctivitis
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10/10. Cicatricial pemphigoid in a 6-year-old child: report of a case and review of the literature.

    Cicatricial pemphigoid has been documented previously in only four patients under the age of 20 years. We report a 6-year-old male who had erosions of the oral and genital mucosa, conjunctivitis, hoarseness, dysphagia, recurrent vomiting, and weight loss. Upper airway obstruction due to a chronically inflamed and scarred epiglottis necessitated tracheostomy. biopsy of tissue from a solitary cutaneous lesion demonstrated a subepidermal bulla. Direct and indirect immunofluorescence confirmed a diagnosis of cicatricial pemphigoid. Therapy was begun consisting of prednisone, 2 mg per kg per day, in combination with dapsone, 2 mg per kg per day. He did not improve until the prednisone dosage was increased to 4 mg per kg per day.
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ranking = 0.5
keywords = conjunctivitis
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