Ligneous (pseudomembranous) inflammation involving the female genital tract associated with type-1 plasminogen deficiency.
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.
Pantanowitz
Liron
L
Department of Pathology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts 02215, USA. lpantano@bidmc.harvard.edu
Bauer
Kenneth
K
Tefs
Katrin
K
Schuster
Volker
V
Balogh
Karoly
K
Pilch
Ben Z
BZ
Adcock
Dorothy
D
Cirovic
Christina
C
Kocher
Olivier
O
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
D010958:Plasminogen; D004247:DNA