title: Periocular Amyloidosis Manifesting as Pseudopemphigoid Treated With Mitomycin C.
abstract: OBJECTIVE
  To describe an unusual cicatrizing manifestation of periocular amyloid and the utility of adjuvant antimetabolite therapy.
  
  
  METHODS
  This is a case report of a 49-year old woman with progressive bilateral upper lid ptosis, who was found to have bilateral inferior forniceal masses with thickening of the conjunctiva. Conjunctival and eyelid biopsies showed evidence of amyloid deposition. Systemic evaluation did not reveal any evidence of systemic amyloidosis. Her blepharoptosis continued to deteriorate, and she underwent bilateral sequential upper lid ptosis correction surgery and debulking procedures. Subsequently, she developed severe and progressive cicatricial conjunctivitis with extensive symblepharon formation in all 4 fornices and restriction of ocular motility, simulating a clinical picture of ocular mucous membrane pemphigoid.
  
  
  RESULTS
  She was treated surgically by division of symblepharon augmented with application of topical mitomycin C intraoperatively. This has been partially successful in freeing up differential movement between her lid and her eye and consequently improving eye protection and lubrication.
  
  
  CONCLUSIONS
  Pseudopemphigoid represents a rare manifestation of periocular amyloid. Risks and benefits of surgical procedures in this context need to be carefully weighed to minimize the risk of secondary complications. Topical mitomycin C may be considered as a relatively safe adjunct in managing the cicatricial component of the disease.
authors.affiliation: *Oculoplastics Department, Royal Eye Infirmary, Derriford Hospital, Plymouth, United Kingdom; and †Histopathology Department, Cambridge University Hospitals NHS Foundation Trust, Cambridge, United Kingdom.
authors.forename: Kaveh
authors.initials: K
authors.lastname: Vahdani
authors.forename: Vladimir T
authors.initials: VT
authors.lastname: Thaller
authors.forename: Giorgio
authors.initials: G
authors.lastname: Albanese
authors.forename: Andrew F
authors.initials: AF
authors.lastname: Dean
mesh_terms: D000477:Alkylating Agents / Q000627:therapeutic use*; D000686:Amyloidosis / Q000175:diagnosis* / Q000188:drug therapy; D003229:Conjunctival Diseases / Q000175:diagnosis* / Q000188:drug therapy; D005141:Eyelid Diseases / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D006801:Humans; D008875:Middle Aged; D016685:Mitomycin / Q000627:therapeutic use*; D010390:Pemphigoid, Benign Mucous Membrane / Q000175:diagnosis* / Q000188:drug therapy
chemical_list: D000477:Alkylating Agents; D016685:Mitomycin