Plasminogen Deficiency as a Rare Cause of Conjunctivitis and Lymphadenopathy

Abstract

Plasminogen deficiency is a rare disorder complicated by the subsequent formation of firm "woody" plaques in the eye (ligneous conjunctivitis) or other mucosal sites as the result of inflammation or trauma. The plaques are composed of fibrinogen, granulation tissue, and inflammatory cells. The findings may be considered nonspecific by the unsuspecting surgical pathologist and delay the appropriate diagnosis. We report the first case of lymph node involvement with characteristic eosinophilic hyaline deposits that are periodic acid Schiff positive, stain dark red with Masson trichrome, and contain fibrinogen as detected by immunofluorescence and describe the longitudinal evolution of this patient's disease over a 15-year period. The differential diagnosis of amorphous hyaline material in lymph node biopsies is discussed.

Orthomolecular nutritional therapy for plasminogen deficiency: report of a case that showed positive results

“…Besides the ocular region, other mucous membranes may be involved, such as oral, nasopharyngeal, gum, trachea and vagina . Several cases of conjunctivitis ligneous and/or ligneous periodontitis were found in association with plasminogen deficiency type I .…”

Lignöz Konjonktivit: Olgu Serisi

“…Konjonktival epitelde ülserasyon veya hiperplastik değişiklikler izlenebilir. 1,6,7 Nadir görülen, genellikle remisyon ve nükslerle uzun bir klinik seyir gösteren lignöz konjonktivitte kabul görmüş etkin bir tedavi protokolü bulunmamaktadır. Oluşan membran ve/veya kitlelerin cerrahi eksizyonu uygun topikal ve/veya sistemik tedavi önlemleri alınmadığında kısa zamanda nüksle sonuçlanmaktadır.…”

“…9 Oluşan psödomembranların immünohistokimyasal incelemesinde T hücrelerinin baskın olduğu infiltrat oluşumu gösterilmiştir. 1,6,9 Siklosporin A'nın T lenfosit proliferasyonunu bloke eden bir immunmodülatör olması terapötik etkinliğini açıklayabilir. Kortikosteroidlerde benzer şekilde immunsupresif etkileriyle lignöz konjonktivitte etkili olmaktadırlar ancak yan etkileri nedeniyle uzun süreli kullanımları uygun değildir.…”

Nutritional treatment for leagnous conjuntivitis: case report and literature review

“…In addition to the ocular region, other mucosas may be involved, such as oral, nasopharyngeal, gums, throat and vagina [7,8] . Several cases of leagnous conjunctivitis and/or leagnous periodontitis were found in association with plasminogen deficiency type I [9,10] . CL is caused by deficiency of plasminogen.…”

“…It plays an important role in the recruitment of macrophages during the inflammatory response, in invasion and metastasis of tumor cells, wound healing, tissue remodeling, growth of neurites and skeletal muscle tissue. [10,11]. The reference values vary according to the laboratory.…”