title: Melkersson-Rosenthal syndrome and acquired C1 inhibitor deficiency.
abstract: BACKGROUND
  Melkersson-Rosenthal syndrome (MRS) is a rare disease whose full-blown form is characterized by orofacial swelling, facial palsy and lingua plicata.
  
  
  OBJECTIVE
  To investigate the complement system as well as its role in patients with MRS.
  
  
  METHODS
  Seven patients presenting at this hospital between November 2002 and May 2003 and meeting the diagnostic criteria according to Hornstein were evaluated retrospectively. The investigations included clinical signs, an analysis of the complement system including levels of CH50, C3, C4, C1 inhibitor (INH) functions and C1-INH antigen detection.
  
  
  RESULTS
  Two female patients showed isolated low levels of functional C1-INH as determined by duplicate tests. Both patients took estrogen-progestin contraceptives.
  
  
  CONCLUSIONS
  Since deficiency in plasma protease C1-INH is known to lead to recurrent angioedema, we hypothesize that low levels of functional C1-INH may have contributed to the orofacial swelling in the 2 patients.
authors.affiliation: Department of Dermatology and Allergy, Tenon Hospital, Paris, France.
authors.forename: F
authors.initials: F
authors.lastname: Masson
authors.forename: S
authors.initials: S
authors.lastname: Barete
authors.forename: V
authors.initials: V
authors.lastname: Frémeaux-Bacchi
authors.forename: H
authors.initials: H
authors.lastname: Szpirglas
authors.forename: S
authors.initials: S
authors.lastname: Agbo-Godeau
authors.forename: O
authors.initials: O
authors.lastname: Chosidow
authors.forename: J C
authors.initials: JC
authors.lastname: Piette
authors.forename: C
authors.initials: C
authors.lastname: Francès
mesh_terms: D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000032:analysis*; D003131:Combined Modality Therapy; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency* / Q000276:immunology; D003176:Complement C3 / Q000032:analysis / Q000276:immunology; D003181:Complement C4 / Q000032:analysis / Q000276:immunology; D015941:Complement Hemolytic Activity Assay; D005260:Female; D006801:Humans; D008297:Male; D008556:Melkersson-Rosenthal Syndrome / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D008875:Middle Aged; D011379:Prognosis; D035583:Rare Diseases; D012189:Retrospective Studies; D018570:Risk Assessment; D012494:Sampling Studies; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index
chemical_list: D015415:Biomarkers; D003174:Complement C1 Inactivator Proteins; D003176:Complement C3; D003181:Complement C4