TİP 1 PLAZMİNOJEN EKSİKLİĞİNE BAĞLI BİR LİGNÖZ KONJONKTİVİT OLGUSUNDA TANI VE TEDAVİ YAKLAŞIMI
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Date
2018
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Access Rights
info:eu-repo/semantics/openAccess
Abstract
Lignöz konjonktivit konjonktivada odunsu yalancı membran oluşumuyla seyreden ve nadir görülen kronik bir hastalıktır. Genellikle otozomal resesif kalıtımlı tip 1 plazminojen eksikliğine bağlıdır. Plazminojen eksikliği fibrinolizis ve yara iyileşmesi bozukluğuna neden olur. Minör travmalar yoğun fibrin ve inflamatuar hücre birikimi gösteren membran oluşumlarını tetikler. Bu membranlar konjonktivada veya vücutta diğer mukozal yüzeylerde ortaya çıkabilir. Tedavide topikal siklosporin, taze donmuş plazma, trombin inhibitörü gibi ajanlar kullanılmasına rağmen nüksler görülebilmektedir. Bu yazıda, 6 yaşında bir lignöz konjonktivit olgusunda tanı ve tedavi yaklaşımını sunmayı amaçladık. Olgumuzda serum plazminojen seviyesi düşüktü (3.4 mg/dl). Eksize edilen membranların histopatolojik incelemesinde eozinofilik amorf materyal birikimi ile yoğun nötrofil baskın iltihabi hücre infiltrasyonu saptandı. Topikal %0.1’lik deksametazon ve %2’lik yüksek konsantrasyonda hazırlanan siklosporin tedavisine iyi yanıt alındı ve 4 yıllık takip süresinde nüks görülmedi. Konjonktivit tablosunun sistemik bir hastalığın göstergesi olabileceği akılda tutulmalı ve şüpheli vakalarda etiyolojik faktörler araştırılmalıdır.
Ligneous conjunctivitis is a rare chronic disease that is characterized with woody pseudomembrane formation in conjunctiva. It is usually due to autosomal recessive inheritance of type 1 plasminogen deficiency. Plasminogen deficiency results in impaired fibrinolysis and wound healing. Minor traumas trigger the formation of intense fibrin and inflammatory cell membrane deposits. These membranes may appear on conjunctiva or other mucosal surfaces in the body. Despite the use of topical cyclosporin, fresh frozen plasma and thrombin inhibitor, recurrences can be observed. In this article, we aimed to present a diagnostic and therapeutic approach for a 6-year-old ligneous conjunctivitis. His serum plasminogen level was low (3.4 mg/dl). Histopathological examination of expelled membranes revealed dense inflammatory cellular infiltrate with neutrophil dominance with eosinophilic amorphous material accumulation. Topical 0.1% dexamethasone and 2% high concentration of cyclosporine treatment were well tolerated and no recurrence was observed at 4 years follow-up. The etiologic factors should be investigated in mindful and suspicious cases where the conjunctivitis table may be indicative of a systemic disease.
Ligneous conjunctivitis is a rare chronic disease that is characterized with woody pseudomembrane formation in conjunctiva. It is usually due to autosomal recessive inheritance of type 1 plasminogen deficiency. Plasminogen deficiency results in impaired fibrinolysis and wound healing. Minor traumas trigger the formation of intense fibrin and inflammatory cell membrane deposits. These membranes may appear on conjunctiva or other mucosal surfaces in the body. Despite the use of topical cyclosporin, fresh frozen plasma and thrombin inhibitor, recurrences can be observed. In this article, we aimed to present a diagnostic and therapeutic approach for a 6-year-old ligneous conjunctivitis. His serum plasminogen level was low (3.4 mg/dl). Histopathological examination of expelled membranes revealed dense inflammatory cellular infiltrate with neutrophil dominance with eosinophilic amorphous material accumulation. Topical 0.1% dexamethasone and 2% high concentration of cyclosporine treatment were well tolerated and no recurrence was observed at 4 years follow-up. The etiologic factors should be investigated in mindful and suspicious cases where the conjunctivitis table may be indicative of a systemic disease.
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Genel ve Dahili Tıp
Journal or Series
Kırıkkale Üniversitesi Tıp Fakültesi Dergisi
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Volume
20
Issue
2
