Ligneous periodontitis and Ehlers-Danlos syndrome.
BACKGROUND
Generalized membranous gingival enlargement due to an accumulation of fibrin deposits associated with severe alveolar bone loss (ligneous periodontitis) is a rare condition, and plasminogen deficiency seems to play a central role in its pathogenesis. However, this condition has not been described in association with syndromes. This article reports a case of ligneous periodontitis in a boy with the classic type of Ehlers-Danlos syndrome (EDS).


METHODS
A 12-year-old white male presented with generalized gingival overgrowth and severe alveolar bone loss. A physical examination revealed clinical signs of EDS (velvety skin with mild hyperextensibility, marked hypermobility of the limb joints, atrophic scars on his knees, and easy bruising), which is associated with a positive family history for joint hypermobility. A biopsy of gingival tissues was submitted for routine histology, hematoxylin and eosin (H&E), and direct immunofluorescence (antifibrinogen). An evaluation of plasminogen activity was also performed.


RESULTS
Histopathology revealed chronic periodontitis with fibrinoid material deposition, and direct immunofluorescence proved to be positive for fibrin. Functional plasminogen was reduced. A conclusive diagnosis of ligneous periodontitis due to plasminogen deficiency associated with the classic type of EDS was rendered.


CONCLUSIONS
Ehlers-Danlos syndrome can be associated with ligneous periodontitis. In the present case, the histologic examination represented an important tool in the differential diagnosis, because it ruled out EDS type VIII as the associated systemic factor to periodontal breakdown.
Department of Pediatric Dentistry and Orthodontics, School of Dentistry, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil. vivigus@bol.com.br
Viviane S S
VS
Pierro
Ramon
R
Vazquez-Sullca
Aurea Simone Barrôso
AS
Vieira
Christina Maeda
CM
Takiya
Gerson
G
Carakushansky
Eduardo J
EJ
Feres-Filho
D016301:Alveolar Bone Loss / Q000209:etiology*; D001706:Biopsy; D002648:Child; D004396:Coloring Agents; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D005337:Fibrin / Q000032:analysis; D019085:Fluorescent Antibody Technique, Direct; D019214:Gingival Overgrowth / Q000209:etiology*; D006801:Humans; D008297:Male; D010518:Periodontitis / Q000209:etiology*; D010958:Plasminogen / Q000172:deficiency
D004396:Coloring Agents; D005337:Fibrin; D010958:Plasminogen