PMC8787558_01
Clinical features at presentation of case 1.. (A-C) Slit lamp images of the right eye showing conjunctival congestion, with symblephara laterally, inferiorly and a macular scar in the inferior cornea. Inferior forniceal shortening is also noted. (D, E) Fluorescein-stained images of the right and left eyes respectively showing punctate keratitis with filaments. (F-H) Slit lamp images of the left eye showing conjunctival congestion, with exuberant granulation tissue and cicatricial changes which are more advanced than those of the right eye.
f1000research-10-120728-g0000.jpg
PMC8787558_01_f1000research-10-120728-g0000.jpg
f1
These fibrotic changes were more pronounced in the lower bulbar and palpebral conjunctiva ( Figure 1).
Timeline of all cases detailing the various immunosuppressive medications and interventions performed.. BE: both eyes, RE: right eye, LE: left eye, IV: intravenous, MP: methyl prednisolone, CP: cyclophosphamide, TPG: tenons patch graft, AMT: amniotic membrane transplantation, SLET: simple limbal epithelial transplant, LSCD: limbal stem cell deficiency, KPro: keratoprosthesis. The numbers in brackets indicate the number of doses, if more than one was given.
f1000research-10-120728-g0001.jpg
PMC8787558_05_f1000research-10-120728-g0001.jpg
f2
Table 1, Figure 2). The visual acuity in the right eye was perception of light while that in the left eye was 20/50.
PMC8787558_02
Timeline of all cases detailing the various immunosuppressive medications and interventions performed.. BE: both eyes, RE: right eye, LE: left eye, IV: intravenous, MP: methyl prednisolone, CP: cyclophosphamide, TPG: tenons patch graft, AMT: amniotic membrane transplantation, SLET: simple limbal epithelial transplant, LSCD: limbal stem cell deficiency, KPro: keratoprosthesis. The numbers in brackets indicate the number of doses, if more than one was given.
f1000research-10-120728-g0001.jpg
PMC8787558_05_f1000research-10-120728-g0001.jpg
f2
Table 1, Figure 2). The visual acuity in the right eye was perception of light while that in the left eye was 20/50.
Clinical features at presentation of case 2.. (A-C) Slit lamp images of the right eye showing conjunctival congestion, with two conjunctival granulomas superiorly (yellow stars) and symblephara inferiorly. (D, E) Fluorescein-stained images of the right and left eyes respectively showing punctate keratitis in the right eye. (F-H) Slit lamp images of the left eye showing conjunctival granulomas (yellow stars), with a symblepharon laterally.
f1000research-10-120728-g0002.jpg
PMC8787558_02_f1000research-10-120728-g0002.jpg
f3
Table 2, Figure 3). The patient was given a pulse dose of IVMP (500 mg) along with topical therapy (full details in Table 1).
Improvement in the clinical features after two doses of pulse IV methyl prednisolone in case 2.. (A-C) Slit lamp images of the right eye showing decrease in congestion with decreased size of the granulomas (yellow stars). (D, E) Fluorescein stained images of the right and left eyes respectively showing a stable surface with no corneal staining. (F-H) Images of the left eye showing decreased inflammation and smaller granulomas (yellow stars).
f1000research-10-120728-g0003.jpg
PMC8787558_02_f1000research-10-120728-g0003.jpg
f4
A significant improvement in the surface inflammation with reduction in the size of the granulomas was noted after 1 week with the topical and systemic immunosuppressants ( Figure 4).
PMC8787558_03
Timeline of all cases detailing the various immunosuppressive medications and interventions performed.. BE: both eyes, RE: right eye, LE: left eye, IV: intravenous, MP: methyl prednisolone, CP: cyclophosphamide, TPG: tenons patch graft, AMT: amniotic membrane transplantation, SLET: simple limbal epithelial transplant, LSCD: limbal stem cell deficiency, KPro: keratoprosthesis. The numbers in brackets indicate the number of doses, if more than one was given.
f1000research-10-120728-g0001.jpg
PMC8787558_05_f1000research-10-120728-g0001.jpg
f2
Table 1, Figure 2). The visual acuity in the right eye was perception of light while that in the left eye was 20/50.
Systemic clinical features at presentation of case 3.. (A, B) Ruptured blisters over the flexor aspect of the thighs (black arrows). (C) Oral ulcers over the hard and soft palate (yellow arrows).
f1000research-10-120728-g0004.jpg
PMC8787558_03_f1000research-10-120728-g0004.jpg
f5
She had oral mucosal ulcers and ruptured blisters over the upper thighs and forearms during the same period ( Figure 5).
Ocular clinical features at presentation of case 3.. (A-C) Slit lamp images of the right eye showing conjunctival congestion, with tarsal scarring superiorly (yellow arrows), limbal thickening (blue arrow) and inferior forniceal shortening. (D, E) Fluorescein-stained images of the right and left eyes respectively showing two epithelial defects in the inferior cornea in the right eye. (F-H) Slit lamp images of the left eye showing tarsal scarring (violet arrows) and inferior forniceal shortening. (I, J) Images of the right and left eye respectively after one year of follow up, showing total limbal stem cell deficiency with a pannus over the cornea. Left eye shows a dermalised ocular surface.
f1000research-10-120728-g0005.jpg
PMC8787558_03_f1000research-10-120728-g0005.jpg
f6
The right eye had two corneal defects inferiorly ( Figure 6, Table 2). Furthermore, the patient did not follow up regularly and one year after the initial presentation, the patient developed total LSCD with a vascularized, scarred cornea and extensive cicatricial changes ( Figure 6).
PMC8787558_04
Timeline of all cases detailing the various immunosuppressive medications and interventions performed.. BE: both eyes, RE: right eye, LE: left eye, IV: intravenous, MP: methyl prednisolone, CP: cyclophosphamide, TPG: tenons patch graft, AMT: amniotic membrane transplantation, SLET: simple limbal epithelial transplant, LSCD: limbal stem cell deficiency, KPro: keratoprosthesis. The numbers in brackets indicate the number of doses, if more than one was given.
f1000research-10-120728-g0001.jpg
PMC8787558_05_f1000research-10-120728-g0001.jpg
f2
Table 1, Figure 2). The visual acuity in the right eye was perception of light while that in the left eye was 20/50.
Periocular blisters around both eyes in case 4.. Some of the blisters have ruptured (yellow arrows) while the others are crusted over (red arrows).
f1000research-10-120728-g0006.jpg
PMC8787558_04_f1000research-10-120728-g0006.jpg
f7
A periocular rash with ruptured blisters was observed in both eyes ( Figure 7).
Ocular clinical features at presentation of case 4.. (A-C) Slit lamp images of the right eye showing thickened distorted mucocutaneous junctions, tarsal scarring superiorly and inferior forniceal shortening with symblephara. (D, E) Fluorescein-stained images of the right and left eyes respectively showing diffuse punctate keratitis in the right eye and a superior epithelial defect in the left eye. (F-H) Images of the left eye showing thickened distorted mucocutaneous junctions and obliteration of the inferior fornix. The bulbar conjunctiva also shows thickened fibrotic changes.
f1000research-10-120728-g0007.jpg
PMC8787558_04_f1000research-10-120728-g0007.jpg
f8
Table 2, Figure 8). The patient received a pulse dose of IVMP (500 mg) with IV cyclophosphamide (500 mg) along with topical medications (full details in Table 1, Figure 2).
PMC8787558_05
Timeline of all cases detailing the various immunosuppressive medications and interventions performed.. BE: both eyes, RE: right eye, LE: left eye, IV: intravenous, MP: methyl prednisolone, CP: cyclophosphamide, TPG: tenons patch graft, AMT: amniotic membrane transplantation, SLET: simple limbal epithelial transplant, LSCD: limbal stem cell deficiency, KPro: keratoprosthesis. The numbers in brackets indicate the number of doses, if more than one was given.
f1000research-10-120728-g0001.jpg
PMC8787558_05_f1000research-10-120728-g0001.jpg
f2
Table 1, Figure 2). The visual acuity in the right eye was perception of light while that in the left eye was 20/50.
Clinical features at presentation of case 5.. (A-C) Slit lamp images of the right eye showing congestion and chemosis with significant collagenolysis in the cornea. (D, E) Fluorescein-stained images of the right and left eyes respectively showing a total epithelial defect in the right eye and a superior epithelial defect in the left eye. (F-H) Images of the left eye showing intense fibrotic changes in the conjunctiva with two granulomas inferiorly (yellow stars). (I) Slit lamp image of the left eye with an extruded Keratoprosthesis. (J) Image of the same eye following a repeat keratoprosthesis, with a skirt amniotic membrane around the prosthesis.
f1000research-10-120728-g0008.jpg
PMC8787558_05_f1000research-10-120728-g0008.jpg
f9
The left eye had a superior corneal epithelial defect with inferior conjunctival granulomas ( Figure 9). However, one year after the surgery, extrusion of the KPro was noted and the patient underwent a repeat KPro ( Figure 9).