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 Table of Contents    
CASE REPORT
Year : 2021  |  Volume : 14  |  Issue : 3  |  Page : 184-186  

Ligneous conjunctivitis exacerbated by Pseudomonas aeruginosa - Diagnostic and therapeutic approach


1 Unit 1, Institute of Ophthalmology, Mayo Hospital, Lahore, Pakistan
2 Department of Medicine, Army Medical College, Rawalpindi, Pakistan
3 Armed Forces Institute of Ophthalmology, Military Hospital, Rawalpindi, Pakistan
4 Al Sadiq-Saad Shaheed Hospital, Rawalpindi, Pakistan

Date of Submission07-Mar-2021
Date of Decision07-Aug-2021
Date of Acceptance12-Aug-2021
Date of Web Publication20-Oct-2021

Correspondence Address:
Dr. Muhammad Abdullah Zahid
32 Lakeside Boulevard, Rowville, Victoria 3178
Pakistan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ojo.ojo_64_21

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   Abstract 


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.

Keywords: Cyclosporine A, heparin, ligneous conjunctivitis, plasminogen deficiency, pseudomembranes, Pseudomonas aeruginosa


How to cite this article:
Tariq M, Zahid MA, Khan TA, Shah HG. Ligneous conjunctivitis exacerbated by Pseudomonas aeruginosa - Diagnostic and therapeutic approach. Oman J Ophthalmol 2021;14:184-6

How to cite this URL:
Tariq M, Zahid MA, Khan TA, Shah HG. Ligneous conjunctivitis exacerbated by Pseudomonas aeruginosa - Diagnostic and therapeutic approach. Oman J Ophthalmol [serial online] 2021 [cited 2021 Dec 8];14:184-6. Available from: https://www.ojoonline.org/text.asp?2021/14/3/184/328612




   Introduction Top


Ligneous conjunctivitis is a type of rare recurrent chronic conjunctivitis which presents with pseudomembranes with a typical wood-like consistency. Approximately 250 cases have been reported to date. The incidence as reported by Schuster and Seregard is estimated to be 16 cases per 1,000,000 people, however, it may be higher in countries where consanguineous marriages are more common.[1] The treatment of ligneous conjunctivitis is very challenging and several studies have reported mixed results. Here, we describe a 3-year-old female patient who presented with bilateral pseudomembranes on bulbar and palpebral conjunctiva. Multidrug-resistant (MDR) Pseudomonas aeruginosa was isolated on culture. This, we believe, has not been reported before. We successfully treated the patient with a regimen of four drugs.


   Case Report Top


A 3-year-old girl presented with the development of bilateral pseudomembranes on the bulbar and palpebral conjunctiva. This was the first time that the patient had developed these pseudomembranes. She was the only child, and thus, a familial link could not be established. However, she was the offspring of a consanguineous marriage. The only positive history was the administration of kohl to her eyes by her parents. Kohl is an ancient eye cosmetic that is widely used in South Asia. A week after the administration of Kohl she gradually developed pseudomembranes bilaterally and these gradually grew over the next month.

On examination, the child was healthy with no other active complaints. Her vitals were normal and several consults from pulmonology and ENT ruled out any other systemic involvement. Her visual acuity could not be ascertained as the child was too young to follow commands and was irritable. As such, eye examination was conducted under general anesthesia. The eyelids were swollen. Grayish white woody pseudomembranes were seen on the bulbar and palpebral conjunctiva. These pseudomembranes also encroached on the outer quadrants of her eyeball. Fundus examination was normal [Figure 1], [Figure 2], [Figure 3].
Figure 1: Pseudomembranes in the right eye

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Figure 2: Pseudomembranes in the left eye

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Figure 3: Postresolution

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Pseudomembranes were also excised from both eyes during this examination, and samples were sent to laboratories for culture and sensitivity and histopathology. A test for plasminogen deficiency was not possible due to the limited finances of the parents. The patient was prophylactically started on topical heparin 5000 IU (½ hourly), topical corticosteroids (½ hourly), and topical tobramycin (2 hourly). However, the pseudomembranes developed again within a week, most prominently on the upper and lower tarsal plates. Histopathology confirmed a diagnosis of ligneous conjunctivitis with underlying fibrinous deposits. Areas of extensive granulation tissue formation were also seen. No evidence of granuloma formation or malignancy was seen. Interestingly culture and sensitivity revealed the growth of P. aeruginosa which was resistant to all the conventional antibiotics except colistin. Following these test results, tobramycin was replaced by topical colistin 0.1% (2 hourly) and topical cyclosporine 2% (2 hourly). It is pertinent to mention that topical colistin was not available commercially and as a result had to be prepared specifically for this patient with the help of the local pharmacy. Following this new regimen of four drugs, namely corticosteroids, heparin, colistin, and cyclosporine 2%, spontaneous resolution of pseudomembranes was seen. Colistin was stopped after 3 weeks and the patient was discharged with the advice of follow-up after 1 month. Upon follow-up, there was new discharge and formation of pseudomembrane on the lower tarsal plate in the left eye, and colistin 0.1% (2 hourly) was restarted again while the doses of the other three medications were reduced. The parents were also counseled on the importance of good eye hygiene and were prescribed acrogel. Another follow-up after 2 months revealed no new growth and as a result topical drug therapy was slowly tapered off. Latest follow-up after 5 months shows no new growth yet.


   Discussion Top


It is largely believed that ligneous conjunctivitis is inherited in an autosomal recessive pattern and that plasminogen deficiency plays a central role in its pathogenesis.[2] Decreased or absent activity of plasmin results in the formation of woody pseudomembranes due to impaired activity of the fibrinolytic system.[1] However, we were unable to test for plasminogen deficiency due to financial constraints and therefore cannot ascertain what the exact cause of ligneous conjunctivitis was in our patient. Recently, this condition has also been hypothesized to be associated with immunoglobulin G 4. Exposure to tranexamic acid[3] has also been implicated as a potential cause. Infectious agents[1] are thought to aggravate the disease. Sahay et al. postulated that Streptococcus pyogenes,  Neisseria More Details gonorrhoeae, Corynebacterium diphtheriae, and adenovirus are the most common organisms that are isolated in most cases of ligneous conjunctivitis.[4] However, in our patient, MDR P. aeruginosa was found to be the aggravating organism. To the best of our knowledge, this organism has not been implicated as an exacerbating factor in the medical literature to date.

The treatment of ligneous conjunctivitis is very challenging as the pseudomembranes tend to recur very quickly. Throughout the past 100 years, various treatment modalities have been used such as tissue plasminogen activator, heterologous serum, plasminogen, antibiotics,[4] heparin,[5] and cyclosporine.[6] It is generally believed that conjunctival surgery induces ligneous conjunctivitis.[7] Treatment with topical plasminogen has largely been effective. DeCock was also successful in treating ligneous conjunctivitis with a combination of corticosteroids and topical heparin,[8] however, other studies have been unable to report positive results with topical heparin alone. Lecame et al.[9] was the first to successfully treat ligneous conjunctivitis using a triple regimen which included corticosteroids, heparin, and cyclosporine. Schuster and Seregard,[1] however, questioned whether the triple regimen was superior to the therapy of corticosteroids and heparin as used by DeCock. This triple regimen was again repeated successfully by Azad et al.[10] in 2009. In our case, we are able to successfully treat our patient with this triple regimen of corticosteroids, heparin, and cyclosporin as well with the only addition of an antibiotic for the MDR organism. As such we have used a combination of four drugs to treat ligneous conjunctivitis. However, as this is a rare disease, further research and case reports are needed to evaluate the efficacy of this four drug regimen.


   Conclusion Top


Ligneous conjunctivitis is a rare disease with only a handful of published cases 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. We have reported in this case report, a MDR P. aeruginosa as the exacerbating organism. Furthermore, we have given merit to the triple regimen of corticosteroids, heparin, and cyclosporin as being effective. We also added antibiotics to target the infectious organism.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Schuster V, Seregard S. Ligneous conjunctivitis. Surv Ophthalmol 2003;48:369-88.  Back to cited text no. 1
    
2.
Schuster V, Hügle B, Tefs K. Plasminogen deficiency. J Thromb Haemost 2007;5:2315-22.  Back to cited text no. 2
    
3.
Song Y, Izumi N, Potts LB, Yoshida A. Tranexamic acid-induced ligneous conjunctivitis with renal failure showed reversible hypoplasminogenaemia. BMJ Case Rep 2014;2014:bcr2014204138.  Back to cited text no. 3
    
4.
Sahay P, Nair S, Maharana PK, Sharma N. Pseudomembranous conjunctivitis: Unveil the curtain. BMJ Case Rep 2019;12:e228538.  Back to cited text no. 4
    
5.
Watts P, Agha SH, Mameesh M, Conor P, Ganesh A, Al-Mujaini A, et al. Fresh frozen plasma (Octaplas) and topical heparin in the management of ligneous conjunctivitis. J AAPOS 2019;23:42-5.e1.  Back to cited text no. 5
    
6.
Holland EJ, Chan CC, Kuwabara T, Palestine AG, Rowsey JJ, Nussenblatt RB. Immunohistologic findings and results of treatment with cyclosporine in ligneous conjunctivitis. Am J Ophthalmol 1989;107:160-6.  Back to cited text no. 6
    
7.
Schwartz GS, Holland EJ. Induction of ligneous conjunctivitis by conjunctival surgery. Am J Ophthalmol 1995;120:253-4.  Back to cited text no. 7
    
8.
De Cock R, Ficker LA, Dart JG, Garner A, Wright P. Topical heparin in the treatment of ligneous conjunctivitis. Ophthalmology 1995;102:1654-9.  Back to cited text no. 8
    
9.
Lecame M, Conan S, Le Jeune V, Dinard BM, Piriou G, Colin J, et al. Treating a ligneous conjunctivitis with false membrane through a local tri-therapy: A glucocorticoid, an anticoagulant and an immunosuppressor. J Pharm Clin 2001;20:25-7.  Back to cited text no. 9
    
10.
Azad N, Zafar S, Khan A. Successful treatment of ligneous conjunctivitis with topical cyclosporine and heparin. J AAPOS 2009;13:519-20.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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