Corneal perforation due to limbal involvement in Sézary syndrome

Abstract

Ocular involvement by cutaneous T-cell lymphoma usually occurs in advanced disease, and carries a poor prognosis. This patient was immunocompromised due to advanced malignancy, and there was a high suspicion of infection as the primary cause of corneal ulceration. This case highlights that it is important to consider direct tumour infiltration as an initiating or contributing factor for corneal ulceration in such patients.

Unusual presentation of fungal keratitis and endogenous endophthalmitis in Sézary syndrome: a case report

“… 9 , 10 Corneal changes were reported in only two cases of Sézary syndrome, neither of which involved fungal keratitis. These included bilateral peripheral ulcerative keratitis (PUK) reported by Maywood et al and a severe case of exposure keratopathy that led to a Pseudomonas corneal ulcer with subsequent perforation despite aggressive therapy described by Livingstone et al 11 , 12 Our case supports these findings, particularly the presence of bilateral cicatricial ectropion and corneal involvement, as shown in Fig. 3 , but represents the first case reported of both Candida keratitis and bacterial endogenous endophthalmitis in the context of Sézary syndrome.…”

“…Of note, the cases of Sézary syndrome and MF reported in the literature that presented with corneal pathology were either due to direct infiltration of atypical T cells from corneal limbus or conjunctiva or had exposure keratopathy secondary to eyelid involvement causing lagophthalmos. 12 , 13 , 14 , 15 Our patient did not have lagophthalmos despite his cicatricial ectropion, and on initial presentation, he had a centrally located dendritiform lesion without corneal thinning. He developed both fungal and bacterial ocular infections in the setting of an advanced hematologic malignancy and significant systemic immunosuppression.…”

Unusual presentation of fungal keratitis and endogenous endophthalmitis in Sézary syndrome: a case report

“… 9 , 10 Corneal changes were reported in only two cases of Sézary syndrome, neither of which involved fungal keratitis. These included bilateral peripheral ulcerative keratitis (PUK) reported by Maywood et al and a severe case of exposure keratopathy that led to a Pseudomonas corneal ulcer with subsequent perforation despite aggressive therapy described by Livingstone et al 11 , 12 Our case supports these findings, particularly the presence of bilateral cicatricial ectropion and corneal involvement, as shown in Fig. 3 , but represents the first case reported of both Candida keratitis and bacterial endogenous endophthalmitis in the context of Sézary syndrome.…”

“…Of note, the cases of Sézary syndrome and MF reported in the literature that presented with corneal pathology were either due to direct infiltration of atypical T cells from corneal limbus or conjunctiva or had exposure keratopathy secondary to eyelid involvement causing lagophthalmos. 12 , 13 , 14 , 15 Our patient did not have lagophthalmos despite his cicatricial ectropion, and on initial presentation, he had a centrally located dendritiform lesion without corneal thinning. He developed both fungal and bacterial ocular infections in the setting of an advanced hematologic malignancy and significant systemic immunosuppression.…”

Bilateral peripheral ulcerative keratitis in a patient with known Sézary syndrome

“… 5 Rather than PUK, the etiology of the ulceration in this case was likely exposure keratopathy secondary to the patient's cicatricial eyelid disease and bacterial superinfection. 5 …”

“…Finally, Livingstone et al reported a Sézary syndrome patient with cicatricial eyelid disease, severe exposure keratopathy, and Pseudomonas corneal ulcer with hypopyon that progressed to perforation despite aggressive topical therapy. 5 The patient underwent enucleation, and pathology revealed limbal infiltration of atypical lymphoid cells consistent with Sézary cells. 5 Rather than PUK, the etiology of the ulceration in this case was likely exposure keratopathy secondary to the patient's cicatricial eyelid disease and bacterial superinfection.…”

“… 5 The patient underwent enucleation, and pathology revealed limbal infiltration of atypical lymphoid cells consistent with Sézary cells. 5 Rather than PUK, the etiology of the ulceration in this case was likely exposure keratopathy secondary to the patient's cicatricial eyelid disease and bacterial superinfection. 5 …”

Unusual presentation of fungal keratitis and endogenous endophthalmitis in Sézary syndrome: a case report

“… 9 , 10 Corneal changes were reported in only two cases of Sézary syndrome, neither of which involved fungal keratitis. These included bilateral peripheral ulcerative keratitis (PUK) reported by Maywood et al and a severe case of exposure keratopathy that led to a Pseudomonas corneal ulcer with subsequent perforation despite aggressive therapy described by Livingstone et al 11 , 12 Our case supports these findings, particularly the presence of bilateral cicatricial ectropion and corneal involvement, as shown in Fig. 3 , but represents the first case reported of both Candida keratitis and bacterial endogenous endophthalmitis in the context of Sézary syndrome.…”

“…Of note, the cases of Sézary syndrome and MF reported in the literature that presented with corneal pathology were either due to direct infiltration of atypical T cells from corneal limbus or conjunctiva or had exposure keratopathy secondary to eyelid involvement causing lagophthalmos. 12 , 13 , 14 , 15 Our patient did not have lagophthalmos despite his cicatricial ectropion, and on initial presentation, he had a centrally located dendritiform lesion without corneal thinning. He developed both fungal and bacterial ocular infections in the setting of an advanced hematologic malignancy and significant systemic immunosuppression.…”

Bilateral peripheral ulcerative keratitis in a patient with known Sézary syndrome

“… 5 Rather than PUK, the etiology of the ulceration in this case was likely exposure keratopathy secondary to the patient's cicatricial eyelid disease and bacterial superinfection. 5 …”

“…Finally, Livingstone et al reported a Sézary syndrome patient with cicatricial eyelid disease, severe exposure keratopathy, and Pseudomonas corneal ulcer with hypopyon that progressed to perforation despite aggressive topical therapy. 5 The patient underwent enucleation, and pathology revealed limbal infiltration of atypical lymphoid cells consistent with Sézary cells. 5 Rather than PUK, the etiology of the ulceration in this case was likely exposure keratopathy secondary to the patient's cicatricial eyelid disease and bacterial superinfection.…”

“… 5 The patient underwent enucleation, and pathology revealed limbal infiltration of atypical lymphoid cells consistent with Sézary cells. 5 Rather than PUK, the etiology of the ulceration in this case was likely exposure keratopathy secondary to the patient's cicatricial eyelid disease and bacterial superinfection. 5 …”

Unusual presentation of fungal keratitis and endogenous endophthalmitis in Sézary syndrome: a case report

“… 9 , 10 Corneal changes were reported in only two cases of Sézary syndrome, neither of which involved fungal keratitis. These included bilateral peripheral ulcerative keratitis (PUK) reported by Maywood et al and a severe case of exposure keratopathy that led to a Pseudomonas corneal ulcer with subsequent perforation despite aggressive therapy described by Livingstone et al 11 , 12 Our case supports these findings, particularly the presence of bilateral cicatricial ectropion and corneal involvement, as shown in Fig. 3 , but represents the first case reported of both Candida keratitis and bacterial endogenous endophthalmitis in the context of Sézary syndrome.…”

“…Of note, the cases of Sézary syndrome and MF reported in the literature that presented with corneal pathology were either due to direct infiltration of atypical T cells from corneal limbus or conjunctiva or had exposure keratopathy secondary to eyelid involvement causing lagophthalmos. 12 , 13 , 14 , 15 Our patient did not have lagophthalmos despite his cicatricial ectropion, and on initial presentation, he had a centrally located dendritiform lesion without corneal thinning. He developed both fungal and bacterial ocular infections in the setting of an advanced hematologic malignancy and significant systemic immunosuppression.…”

Bilateral peripheral ulcerative keratitis in a patient with known Sézary syndrome

“… 5 Rather than PUK, the etiology of the ulceration in this case was likely exposure keratopathy secondary to the patient's cicatricial eyelid disease and bacterial superinfection. 5 …”

“…Finally, Livingstone et al reported a Sézary syndrome patient with cicatricial eyelid disease, severe exposure keratopathy, and Pseudomonas corneal ulcer with hypopyon that progressed to perforation despite aggressive topical therapy. 5 The patient underwent enucleation, and pathology revealed limbal infiltration of atypical lymphoid cells consistent with Sézary cells. 5 Rather than PUK, the etiology of the ulceration in this case was likely exposure keratopathy secondary to the patient's cicatricial eyelid disease and bacterial superinfection.…”

“… 5 The patient underwent enucleation, and pathology revealed limbal infiltration of atypical lymphoid cells consistent with Sézary cells. 5 Rather than PUK, the etiology of the ulceration in this case was likely exposure keratopathy secondary to the patient's cicatricial eyelid disease and bacterial superinfection. 5 …”