title: Ligneous (pseudomembranous) inflammation involving the female genital tract associated with type-1 plasminogen deficiency.
abstract: Ligneous (pseudomembranous) inflammation of the female genital tract is a rare and unusual condition characterized by extensive subepithelial fibrin deposition and associated inflammation. Ligneous inflammation in extragenital sites, predominantly the conjunctiva, has been linked to plasminogen deficiency. Individuals with plasminogen deficiency are unable to remove fibrin deposited in injured mucosal tissue. Severe systemic ligneous inflammation involving the female genital tract that is linked to hypoplasminogenemia has not previously been described. We present a patient with ligneous inflammation of her genital, middle ear, and oral mucosa that was associated with type-1 homozygous plasminogen deficiency due to a novel missense mutation in the plasminogen gene.
authors.affiliation: Department of Pathology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts 02215, USA. lpantano@bidmc.harvard.edu
authors.forename: Liron
authors.initials: L
authors.lastname: Pantanowitz
authors.forename: Kenneth
authors.initials: K
authors.lastname: Bauer
authors.forename: Katrin
authors.initials: K
authors.lastname: Tefs
authors.forename: Volker
authors.initials: V
authors.lastname: Schuster
authors.forename: Karoly
authors.initials: K
authors.lastname: Balogh
authors.forename: Ben Z
authors.initials: BZ
authors.lastname: Pilch
authors.forename: Dorothy
authors.initials: D
authors.lastname: Adcock
authors.forename: Christina
authors.initials: C
authors.lastname: Cirovic
authors.forename: Olivier
authors.initials: O
authors.lastname: Kocher
mesh_terms: D004247:DNA / Q000737:chemistry / Q000235:genetics; D004427:Ear Diseases / Q000209:etiology / Q000473:pathology; D005260:Female; D005831:Genital Diseases, Female / Q000209:etiology / Q000473:pathology*; D005882:Gingival Diseases / Q000209:etiology / Q000473:pathology; D006801:Humans; D007249:Inflammation / Q000209:etiology / Q000473:pathology; D008875:Middle Aged; D020125:Mutation, Missense; D010958:Plasminogen / Q000172:deficiency* / Q000235:genetics; D016133:Polymerase Chain Reaction; D018807:Polymorphism, Single-Stranded Conformational
chemical_list: D010958:Plasminogen; D004247:DNA