Cases reported "Conjunctivitis"

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1/4. Chronic cicatrizing conjunctivitis in a patient with epidermolysis bullosa acquisita.

    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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ranking = 1
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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2/4. Dermolytic (dystrophic) epidermolysis bullosa inversa.

    We treated four patients with an inverse form of recessive dermolytic (dystrophic) epidermolysis bullosa. The distinguishing features of the disease are (1) early generalized skin involvement with blisters and erosions that heal with superficial, atrophic scars; (2) persistence into adulthood, although milder; (3) severity in flexural areas, especially the inguinal folds, perineum, axillae, submammary area, posterior and lateral aspects of the neck, and often the lower parts of the abdomen and back; (4) normal stature and general development; (5) severe oral and esophageal mucosal involvement; (6) normal teeth; (7) normal or minimally involved fingernails, but mild to moderately dystrophic or atrophic toenail changes; and (8) microscopic findings similar to those of the Hallopeau-Siemens form of epidermolysis bullosa.
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ranking = 0.099888045597356
keywords = epidermolysis bullosa, epidermolysis, bullosa
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3/4. The spectrum of epidermolysis bullosa acquisita.

    A patient has an acquired, scarring, bullous eruption. The severity of the cutaneous, ocular, esophageal, and laryngeal scarring was suggestive of cicatricial pemphigoid or the severely dystrophic forms of epidermolysis bullosa. Clinical features and histologic and immunofluorescence and electron microscopic study led to the diagnosis of epidermolysis bullosa acquisita. The spectrum of epidermolysis bullosa acquisita includes cases that closely approximate the severity of disease previously recognized only for the recessive form of epidermolysis bullosa dystrophica.
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ranking = 0.86557412071813
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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4/4. 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). DESIGN: case reports. PARTICIPANTS: Two patients. INTERVENTION: epidermolysis bullosa acquisita associated with cicatrizing conjunctivitis. MAIN OUTCOME MEASURES: 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.
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ranking = 0.28375740205895
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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