Ligneous Conjunctivitis: Complication of Inborn Plasminogen Deficiency
(a Case Report)
Dostálek M.1, Krásný J.2, Špička I.3, Šach J.4
1Ortoptická část, Oční oddělení, Nemocnice, Litomyšl, primář MUDr. Vladimír Liška, Ph.D. 2Oční klinika FNKV, Praha, přednosta prof. MUDr. Pavel Kuchyňka, CSc. 31. interní klinika VFN, Praha, přednosta prof. MUDr. Pavel Klener, DrSc. 4Ústav patologie 3. LF UK, Praha, přednosta doc. MUDr. V. Mandys, CSc. |
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Summary:
Authors refer about detailed analysis of ten years follow up of a child patient
with ligneous conjunctivitis. They document presence of all typical eye
findings (especially recurrent formation of granulomatous pseudotumors in
affected mucous membrane) related to this rare pseudomembranous
conjunctivitis. The clinical picture of the disease includes plasminogen
deficiency, a factor newly considered as primary cause of the disease. The
diagnosis was confirmed histologicaly by repeated probatory excisions.
Pseudomembranous inflammation with mixed inflammatory infiltrate
containing large amount of elements of chronical as well as acute
inflammatory reaction is typical. Granuloma with the accumulation of the PASpositive
amorphous matrix with high content of the fibrin and with the
network of newly formed vessels is the component of the inflammatory picture.
The treatment was based on successively discovered knowledge of the possible
etiopathogenesis of the disease. Only the ninth, last surgical procedure
induced longer-term remission, which lasts sixteen months until now. The
granuloma excision was combined with the mitomicin application on the
wound surface and a conjunctivoplasty. During the postoperative period, the
ointment with heparin, corticosteroid and antibiotic was applied. The purpose
of the mitomicin use is to slow down the fibroproliferative reparative reaction.
Covering of the wound surface decreases the number of microtraumas on the
uneven postoperative scleral surface. Heparin in the interstitial tissue of the
wound surface blocks the conversion of fibrinogen to fibrin.
Key words:
ligneous conjunctivitis, plasminogen, heparin, mitomycin C,
aberrant healing, pseudomembranous inflammation
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