title: Ocular mucous membrane pemphigoid after Lyell syndrome: occasional finding or predisposing event?
abstract: OBJECTIVE
  Ocular mucous membrane pemphigoid (OMMP) is an autoimmune disease involving the eye and characterized by subepithelial detachment resulting from an immunologic reaction against conjunctival basal membrane zone (BMZ) antigens. Lyell syndrome (LS) is a drug-induced, T cell-mediated, cytotoxic reaction involving the mucocutaneous areas. Two patients with LS are presented in whom OMMP developed.
  
  
  METHODS
  Report of 2 cases.
  
  
  METHODS
  Two male patients, 80 and 60 years old, with persistent corneal ulcerations, corneal melting, and inflammation some months after an LS episode.
  
  
  METHODS
  Conjunctival biopsy samples were obtained to perform direct immunofluorescence (DIF) and histologic analyses. Indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assay (ELISA) also were performed.
  
  
  METHODS
  Immunodeposit findings on the conjunctival BMZ obtained by DIF and IIF, inflammatory infiltration of the corneoconjunctival samples studied by histologic analysis, and autoantibodies of patient sera directed against BMZ antigens tested by ELISA.
  
  
  RESULTS
  Direct immunofluorescence analyses showed immunoglobulin G and complement 3 component deposits along the BMZ in a linear pattern. Histologic analysis revealed the presence of eosinophils, neutrophils, and mast cells with fibrin deposition in the substantia propria of both patients; the data confirmed the clinical suspicion of OMMP. The IIF and ELISA results were negative.
  
  
  CONCLUSIONS
  Chronic eye surface injury associated with LS may promote autoimmunization against ocular epithelial BMZ antigens, playing a strategic role in the subsequent onset of OMMP. The occurrence of OMMP after LS could be an occasional finding, or conversely, LS could be an underestimated predisposing factor in the development of OMMP.
authors.affiliation: Department of Dermatology, Universitá del Sacro Cuore, Rome, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Fania
authors.forename: Maria I
authors.initials: MI
authors.lastname: Giannico
authors.forename: Romina
authors.initials: R
authors.lastname: Fasciani
authors.forename: Anna
authors.initials: A
authors.lastname: Zampetti
authors.forename: Simone
authors.initials: S
authors.lastname: Ambrogio
authors.forename: Emilio
authors.initials: E
authors.lastname: Balestrazzi
authors.forename: Claudio
authors.initials: C
authors.lastname: Feliciani
mesh_terms: D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood; D001324:Autoantigens / Q000276:immunology; D001485:Basement Membrane / Q000276:immunology; D001706:Biopsy; D003229:Conjunctival Diseases / Q000175:diagnosis / Q000209:etiology*; D004797:Enzyme-Linked Immunosorbent Assay; D019085:Fluorescent Antibody Technique, Direct; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D008297:Male; D008875:Middle Aged; D010390:Pemphigoid, Benign Mucous Membrane / Q000175:diagnosis / Q000209:etiology*; D012307:Risk Factors; D013262:Stevens-Johnson Syndrome / Q000150:complications*
chemical_list: D001323:Autoantibodies; D001324:Autoantigens