- Cornea. 2004 Nov;23(8):819-22.
Ocular Immunology and Uveitis Service, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA 02114, USA.
PURPOSE: To report the case of a patient developing fungal keratitis in the context of uncontrolled ocular cicatricial pemphigoid (OCP), which, despite intravenous immunoglobulin (IVIg) and other immunomodulatory therapy, progressed to end-stage pemphigoid, with corneal opacification, ankyloblepharon, and xerosis. Keratoprosthesis (KPro) restored functional vision for the patient. METHODS: A 39-year-old man presented with uncontrolled CP and corneal ulcer in the left eye. Conjunctival biopsy diagnosed OCP; corneal scraping and biopsy diagnosed the cause of the corneal ulcer. OCP was treated with systemic steroids, immunosuppressive drugs, and IVIg. Visual rehabilitation was accomplished with Ahmed valve and a type II Dohlman KPro. RESULTS: Immunohistology of the biopsied conjunctiva showed IgG at the epithelial basement membrane zone, confirming the clinical diagnosis of OCP. Microbiologic studies of the corneal biopsy specimen were negative for Acanthamoeba and herpes but positive for Aspergillus niger. The patient's keratomycosis resolved with topical antifungal therapy. Treatment with Dapsone, intravenous-pulse steroid, oral cyclophosphamide, and intravenous immunoglobulin (IVIg) failed to control the OCP, with resultant complete conjunctivization of the cornea. Keratoprosthesis improved the patient's visual acuity from hand movements to 20/20. CONCLUSIONS: Patients with uncontrolled OCP are at increased risk of corneal infection. The difficulty in diagnosing keratomycosis and the relatively rare occurrence of OCP explain the uniqueness of our reported case. OCP may progress to "end-stage" disease despite therapy. Keratoprosthesis can restore vision in selected otherwise seemingly hopeless cases.
PMID: 15502484 [PubMed - indexed for MEDLINE]
Saturday, February 3, 2007
Ocular cicatricial pemphigoid, keratomycosis, and intravenous immunoglobulin therapy.
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Hi! I'm glad to stop by your site and know more about ocular cicatricial pemphigoid. Keep it up! This is a good read. I will be looking forward to visit your page again and for your other posts as well. Thank you for sharing your thoughts about ocular cicatricial pemphigoid.
Cicatricial pemphigoid has been referred to by a variety of designations based largely on its site of involvements, with examples of such terminology including "desquamative gingivitis," "ocular pemphigus," and "benign mucous membrane pemphigoid." However, currently "...such designations are thought to be confusing or somewhat misleadings (e.g., pemphigus in this context is a misnomer , and this disorder is hardly benign given the extent of morbidity it can cause)."
patient's immune system, with the white blood cells becoming "confused" and beginning to attack not only germs but also part of the patient's own body. In rheumatoid arthritis, for example, the white blood cells become confused and begin to attack tissue in the patient's joints. In pemphigoid, the white blood cells attack skin and mucous membrane, particularly mucous membranes of the mouth, eyes, nose, throat, vagina, and rectum. Patients may or may not have more than one site affected by the disease, but 70% of the patients with cicatricial pemphigoid have eye involvement. And because this is a systemic autoimmune disease, it cannot be successfully treated, long-term, with simple topical (drops) therapy. It must be treated systemically, and typically with strategies that "cool down" the immune system, i.e., suppress the immune system sufficiently to stop the autoimmune process. Suppressing the immune system sufficiently to stop the autoimmune process for a short period (one to five years) is usually sufficient to enduce permanent remission of the disease. If this is not done, then continued inflammation, continued scarring, and eventual blindness in both eyes usually occurs.
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- The Ocular Cicatricial Pemphigoid
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