This case report explores the rare association of recurrent meningitis, hydrocephalus, ligneous conjunctivitis, and congenital plasminogen deficiency in a term baby boy. Born at 39 weeks with a history of hydrocephalus, the neonate later developed ligneous conjunctivitis and a serious bout of meningitis. Genetic analyses confirmed a homozygous mutation in the PLG gene, indicative of congenital plasminogen deficiency. Despite intensive treatment, including a ventriculoperitoneal shunt for hydrocephalus and intravenous antibiotics for meningitis, the child succumbed to upper airway obstruction before reaching one year of age. This report underscores the medical complexity and severity of these interconnected conditions and advocates for further research to understand the interplay between them. Although this study is limited by its single-case nature and is not generalizable, it emphasizes the necessity for early recognition and a multidisciplinary treatment approach for better patient outcomes.

BACKGROUND: Ligneous conjunctivitis (LC), a rare chronic conjunctivitis linked to plasminogen deficiency, poses challenges in research and management due to its complexity. This case report details the combined medical and surgical approach in managing LC in a 1.5-year-old female with congenital hydrocephalus, plasminogen deficiency, and glaucoma.
METHODS: The patient\'s LC required a comprehensive treatment plan involving systemic and topical therapies, surgical intervention, and fresh frozen plasma (FFP) infusions via a central line.
CONCLUSIONS: Managing LC necessitates a comprehensive, individualized approach due to its rarity and lack of standardized treatments. Various therapies have shown promise, but their availability, cost, and potential side effects vary. FFP infusions played a crucial role in managing plasminogen deficiency, but the potential complications associated with central lines must be considered. Ongoing clinical trials aim to improve LC treatment, but until results are available, clinicians must rely on existing evidence and clinical judgment.
CONCLUSIONS: This case underscores the complexity of managing LC and the significance of adopting an individualized treatment strategy. Regular follow-ups are crucial for flexibility and adaptability in treatment plans to address potential recurrences. Further research is necessary to validate these findings and assess the long-term effectiveness of the combined approach.

BACKGROUND: Ligneous Conjunctivitis (LC) is the most common clinical manifestation of Type I Plasminogen deficiency (T1PD; OMIM# 217090), and it is characterized by the formation of pseudomembranes (due to deposition of fibrin) on the conjunctivae leading to progressive vision loss. In past times, patients with LC were treated with surgery, topical anti-inflammatory, cytostatic agents, and systemic immunosuppressive drugs with limited results (Blood 108:3021-3026, 2006, Ophthalmology 129:955-957, 2022, Surv Ophthalmol 48:369-388, 2003, Blood 131:1301-1310, 2018). The surgery can also trigger the development of membranes, as observed in patients needing ocular prosthesis (Surv Ophthalmol 48:369-388, 2003). Treatment with topical purified plasminogen is used to prevent pseudomembranes formation (Blood 108:3021-3026, 2006, Ophthalmology 129:955-957, 2022).
METHODS: We present the case of a sixteen-year-old girl with LC with severe left eye involvement. We reported the clinical conditions of the patient before and after the use of topical plasminogen eye drops and described the treatment schedule allowing the surgical procedure for the pseudomembranes debulking and the subsequent use of ocular prosthesis for aesthetic rehabilitation.
CONCLUSIONS: The patient showed a progressive response to the topical plasminogen, with a complete absence of pseudomembrane formation at a twelve-year follow-up, despite using an ocular prosthesis.

BACKGROUND: Ligneous conjunctivitis (LC) is a rare inflammatory lesion of the conjunctiva with an unknown etiology. It is easily confused with conjunctiva lymphoma or other diseases in clinical diagnosis, and the lesion is very difficult to treat.
METHODS: We presented a 41-year-old female patient presented with bilateral conjunctival masses for more than six months. The patient had no contributory history of ocular trauma, family history of tumor and drug allergy. Taking the patient\'s clinical and pathological features together, we considered this was a case of IgG4 + LC. Completely surgical resection combined with local glucocorticoid treatment might be effective.
CONCLUSIONS: This is a very rare case report of immunoglobulin G4 positive LC with only one published case in literature. The typical manifestations of LC are with the appearance of a hard, fibrin-rich, woody pseudomembranous lesion. A large number of lymphocyte and plasma cell are infiltrated in the pathological tissue. Inflammation of LC may cause immune abnormalities, resulting in IgG4 increasing.

Ligneous conjunctivitis is a very rare form of pseudomembranous conjunctivitis with few published cases in literature. We aim to describe the ocular findings and treatment in an infant with ligneous conjunctivitis resembling preseptal cellulitis on presentation.
Case report of a 3-month-old girl who was referred to a tertiary centre for ophthalmic assessment due to progressive eyelid oedema with no response to initiated topical and systemic antibiotics. Ethical approval has been achieved from the local ethics committee of the Ghent University Hospital and informed consent has been obtained from the parents of the child.
Examination under general anaesthesia showed multiple, wood-like fibrinous pseudomembranes, originating from the conjunctiva, consistent with ligneous conjunctivitis. After careful removal of the coagulated exudate covering the cornea, a central corneal epithelial defect was evident without stromal infiltration. Histopathologic examination confirmed the predominance of fibrin within the pseudomembranes. Plasminogen activity was below the normal range. Genetic analysis did not identify a pathogenic variant in the PLG gene. The corneal epithelium re-epithelialised during the following days and the conjunctival lesions gradually subsided over the ensuing weeks whilst continuing heparin-containing artificial tears.
A high level of suspicion is warranted in atypical cases of preseptal cellulitis which show no response to antibiotic treatment. Particularly in young children, examination under general anesthesia is warranted to allow diagnosis of rare causes of secondary eyelid oedema. We report an infant with unilateral ligneous conjunctivitis who responded well to topical, commercially-available heparin-containing artificial tears treatment. This approach is an effective and easy first-line treatment option in this condition, particularly in milder phenotypes.

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BACKGROUND: Ligneous conjunctivitis is a rare form of chronic pseudomembranous conjunctivitis which usually starts during infancy. We report on an unsual case of recurrent ligneous conjunctivitis after cataract surgery in a 67-year-old male patient.
METHODS: The equipment used for the slit-lamp images was a Haag Streit slit lamp BX900 Sn 00,406 with 16 × magnifications. The used batch number of the camera was sn00406 and the software was from the company CCS Pawlowski Merge Eye. There were no filters used. The images were saved with a resolution of 300 DPI. Neither downstream nor averaging was used to enhance the resolution of the image in the case presentation section or the figure legend. The equipment used for the cross-sectional histologic images was a Zeiss Axioskop 40 microscope with an objective lens Zeiss A-Plan × 20/0.45 (Zoom 6.3 × TV 2/3″″C). The used camera was AxioCam MRc5 and the software was ZEN 3.2. The cross-sectional histologic images were saved with a resolution of 2584 × 1936 Pixels. Neither downstream nor averaging was used to enhance the resolution of the image in the case presentation section or the figure legend.
METHODS: This is a rare case report of ligneous conjunctivitis in a 67-year-old male patient who presented a recurrent conjunctival granuloma after five excisions following cataract surgery in his left eye. We performed a tumor excision with free conjunctival autograft. The histology showed a fibrin crust including macrophages, granulocytes, lymphocytes, and reactively altered squamous cell nests. These findings were consistent with a ″pseudomembrane in conjunctivitis lignosa″. We administered a topical combination of plasminactivator, heparin, cortisone and cyclosporine.
CONCLUSIONS: This treatment with the combination of plasminactivator, heparin, cortisone and cyclosporine has proven to be effective in preventing the recurrence of ligneous conjunctivitis.

Ligneous conjunctivitis (LC) is a rare form of pseudomembranous chronic conjunctivitis caused by a deficiency in plasminogen activity. Due to its rarity, little is known about this disorder. We hereby report a case of ligneous conjunctivitis, describing the clinical findings, the biological diagnosis and the treatment of this rare disease.

Ligneous conjunctivitis is a rare disease in which pseudomembranes develop on the mucosal surfaces of the eye. Only a handful of cases have been reported in the past 100 years. Although plasminogen deficiency is largely implicated in the pathogenesis of this condition, infectious agents are also thought to play a role in worsening the disease. Treatment is usually challenging. We present a case of 3-year-old female in whom a multidrug-resistant Pseudomonas aeruginosa was isolated from the culture of the pseudomembranes. To the best of our knowledge, this is the first time P. aeruginosa has been implicated as an exacerbation factor. Furthermore, we have given merit to the triple regimen of corticosteroids, heparin, and cyclosporine as being effective. We also added antibiotics to target the infectious organism.

Ligneous conjunctivitis is a rare form of chronic, recurrent conjunctivitis characterized by wood-like, fibrinous pseudomembranes, which may be associated with systemic disease manifestations. It has been associated with congenital plasminogen (PLG) deficiency that is inherited with an autosomal recessive pattern due to mutations in the PLG gene and a variety of other genes, leading to disturbed wound healing. In this case report, we present the clinical, laboratory, and histopathological findings of a 36-year-old female patient who presented at the ophthalmology department with complaints of redness, irritation for the previous few weeks, and appearance of membranous lesions mainly on the tarsal conjunctivae. During biomicroscopic examination we found thick, yellowish-white pseudomembranes, and conjunctival proliferation with ligneous induration on the conjunctiva, located on the upper eyelids. Histopathological evaluations showed up ligneous conjunctivitis and laboratory evaluation confirmed a severe plasminogen deficiency (PLG < 2%). The patient was treated with topical fresh frozen plasma (FFP), topical steroids, heparin eye drops, and artificial tear drops daily, without systemic therapy.