title: Ocular cicatricial pemphigoid, keratomycosis, and intravenous immunoglobulin therapy.
abstract: OBJECTIVE
  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.
authors.affiliation: Ocular Immunology and Uveitis Service, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA 02114, USA.
authors.forename: Barbara
authors.initials: B
authors.lastname: Iaccheri
authors.forename: Manolette
authors.initials: M
authors.lastname: Roque
authors.forename: Tito
authors.initials: T
authors.lastname: Fiore
authors.forename: Thekla
authors.initials: T
authors.lastname: Papadaki
authors.forename: Benjamin
authors.initials: B
authors.lastname: Mathew
authors.forename: Stefanos
authors.initials: S
authors.lastname: Baltatzis
authors.forename: Barry
authors.initials: B
authors.lastname: Emara
authors.forename: A C
authors.initials: AC
authors.lastname: Tokarewicz
authors.forename: C Stephen
authors.initials: CS
authors.lastname: Foster
mesh_terms: D000328:Adult; D000666:Amphotericin B / Q000627:therapeutic use; D000935:Antifungal Agents / Q000627:therapeutic use; D001228:Aspergillosis / Q000150:complications* / Q000188:drug therapy / Q000382:microbiology; D001234:Aspergillus niger / Q000302:isolation & purification; D001327:Autoimmune Diseases / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D003231:Conjunctivitis / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D003315:Cornea / Q000382:microbiology; D003318:Corneal Opacity / Q000209:etiology / Q000601:surgery; D003320:Corneal Ulcer / Q000150:complications* / Q000188:drug therapy / Q000382:microbiology; D015821:Eye Infections, Fungal / Q000150:complications* / Q000188:drug therapy / Q000382:microbiology; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D010390:Pemphigoid, Benign Mucous Membrane / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D019736:Prostheses and Implants; D019919:Prosthesis Implantation
chemical_list: D000935:Antifungal Agents; D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D000666:Amphotericin B