bs_bs_banner
Letters to the Editor
191
Rare case of Langerhans cell histiocytosis of the conjunctiva Langerhans cells are specific antigen presenting cells normally found in the basal layer of the epidermis, squamous epithelia, hair follicles and apocrine/sebaceous glands. Langerhans cell histiocytosis (LCH) is a rare condition characterized by neoplastic proliferation of Langerhans cells and usually affects men and children.1 Langerhans cell histiocytosis lesions are usually unifocal and typically involve the skull, pelvis and femur. Ocular or orbital involvement is very rare. We present a case of rare ocular LCH in a 22-year-old male with conjunctival Langerhans cell histiocytosis. The patient initially presented with a year-long history of temporal injection and irritation of the right eye. He reported no visual deterioration and had previously tried multiple types of eye lubricants without any improvement in symptoms. The patient had recently immigrated to Australia from Afghanistan and reported no other ophthalmic history, medical history or surgical history. He took no regular medications and had no allergies. He reported feeling otherwise well with no other systemic symptoms. On initial examination, visual acuity was 6/5 in both eyes with normal intraocular pressures and no sign of proptosis. Anterior segment examination showed injected temporal conjunctiva and sclera in the right eye. There was no blanching of the injection with 10% topical phenylephrine and no associated fluorescein uptake. Otherwise the eyes were quiet, with clear corneas and no sign of intraocular inflammation. Posterior segment examination was unremarkable bilaterally. The patient was initially treated with topical corticosteroids and oral ibuprofen with a working diagnosis of early nodular non-necrotizing scleritis. Extensive blood testing involving full blood count, electrolytes, liver function tests, inflammatory markers, rheumatoid factor, ANA, C-ANCA/P-ANCA, syphilis, QuantiFERON Gold (Cellestis, Ltd. Carnegie, Australia) and ACE were all normal. Chest X-ray was also normal. Over the next month, the patient’s irritation and right eye injection only settled slightly. Further examination revealed a raised 3 mm diameter fibrovascular mass with feeder vessels within the now reduced right temporal injection (Fig. 1). The mass appeared to be within the conjunctiva with no evidence of any deeper invasion. Excisional biopsy was recommended and undertaken 3 weeks later. The conjunctival lesion was easily excised with adequate macroscopic margins, and cryotherapy was applied to the edges of the conjunctival defect. Histopathology showed aggregates of Langerhans cells with a surrounding infiltrate of eosinophils and lymphocytes. Immunohistochemistry confirmed positive staining for CD1a and S100. Features were consistent with conjunctival LCH. Margins of the specimen were clear.
Competing/conflicts of interest: No stated conflict of interest. Funding sources: No stated funding sources.
Figure 1. Anterior segment photography of the conjunctival Langerhans cell histiocytosis lesion prior to excision. The patient’s eye settled postoperatively with no further irritation or injection and was subsequently referred to haematology. A detailed systemic examination including Computed Tomography (CT) head, chest, abdomen and pelvis, positron emission tomography scan, magnetic resonance imaging head and bone marrow aspirate and trephine showed no evidence of systemic LCH, and a final diagnosis of unifocal conjunctival LCH was made. Conjunctival LCH has only been reported six times previously, three times at the conjunctival limbus,1–3 twice at the caruncle4,5 and once on the epibulbar conjunctiva.6 Although this is a very rare condition, LCH should be recognized as a possible malignant cause of a conjunctival lesion with potentially serious systemic manifestations, and thus should be considered in the differential diagnosis of any conjunctival lesion.
Thomas P Moloney MBBS, Mohammad Ghaznawi MBBS, Sean Cheng MBBS and Andrew Apel FRANZCO Department of Ophthalmology, Princess Alexandra Hospital, Brisbane, Queensland, Australia Received 23 June 2014; accepted 25 June 2014.
REFERENCES 1. Saxena T, Kumar K, Sen S, Tandon R. Langerhans cell histiocytosis presenting as a limbal nodule in an adult patient. Am J Ophthalmol 2004; 138: 508–10. 2. Bakhshi S, Nongpiur ME. Recurrence of Unifocal Limbal Langerhans cell histiocytosis with partial response to chemotherapy and prolonged remission. Pediatr Blood Cancer 2011; 56: 687–8. 3. Kanavi MR, Javadi F, Javadi M, Faramarzi A. Unifocal Langerhans cell histiocytosis stimulating a limbal papilloma. J Ophthalmic Vis Res 2012; 7: 240–3. 4. Stanowsky A, Krey HF, Wagner T. Histiocytosis X (eosinophiles Granulom) der Karunkel. Klin Mbl Augenheilk 1991; 199: 359–61.
© 2014 Royal Australian and New Zealand College of Ophthalmologists
bs_bs_banner
192
Letters to the Editor
5. Kiratli H, Kocabeyoglu S, Saglam A, Soylemezoglu F. Langerhans cell histiocytosis of the caruncle. Clin Experiment Ophthalmol 2007; 35: 661–3. 6. Melamud A, Efrat M, Sova Y, Hod Y, Geyer O. Epibulbar nodule as a symptom of Langerhans cellhistiocytosis. Arch Ophthalmol 2002; 120: 1400–1.
Inappropriate use of topical chloramphenicol results in vision loss Microbial keratitis is an ophthalmic emergency. We present two cases of microbial keratitis in which inappropriate chloramphenicol use delayed diagnosis and hence prompt management. For one patient, the result was loss of the eye, and for the other, a child, lifelong reduced vision from corneal scarring. The first case was a 69-year-old woman who presented with a three-week history of a red, watery right eye and gradually reducing vision. She had been self-treating with chloramphenicol drops and ointment, obtained from her pharmacist without medical consultation. Her history was of keratoconjunctivitis sicca secondary to rheumatoid arthritis, treated with lubricants and topical chloramphenicol when required for ‘red eye’. Her presenting vision was reduced to 6/45 in the affected eye. She had a right corneal abscess, with a slow leak from a corneal perforation. Following corneal scraping, glue was applied and intensive topical fortified cephalothin and gentamicin commenced. Streptococcus pneumonia was isolated from the corneal scrape. Over 48 h, her vision decreased to light perception, and anterior chamber activity increased. Gentamicin was ceased and topical ofloxacin commenced. The organism was then reported to be multi-resistant, and oral ciprofloxacin was commenced. However, despite appropriate management, the infection progressed to involve the vitreous and then sclera, and the eye required evisceration at day five. In the second case, a 16-year-old girl orthokeratology contact lens wearer presented with a two-week history of cloudy left vision and nine days of eye pain. She had worn night-time orthokeratology lenses for three years. At the onset of her symptoms, her optometrist had advised her to use chloramphenicol eye drops. After one week of worsening symptoms, she sought treatment from an ophthalmologist. Her presenting vision was 6/15 in the affected eye. She had a central corneal infiltrate with a surrounding ring
Nikki McDerby MNedSci MPharm,1 Stephanie L Watson PhD FRANZCO,2,3 Dana Robaei PhD FRANZCO2,4 and Mark Naunton BPharm(Hons) PhD1 1 Faculty of Health, Discipline of Pharmacy, University of Canberra, Canberra, Australian Capital Territory, 2Save Sight Institute, University of Sydney, 3Sydney Eye Hospital, and 4Department of Ophthalmology, Westmead Hospital, Sydney, New South Wales, Australia Received 19 October 2014; accepted 26 October 2014.
REFERENCES
Competing/conflicts of interest: No stated conflict of interest. Funding sources: Dr Robaei is funded by an NHMRC Early Career Fellowship (APP1073846). Professor Watson is funded by an NHMRC Career Development Fellowship (APP1050524). The two cases are part of the Serious Ocular Infections Project (SOIP), funded by the Sydney Eye Hospital Foundation.
infiltrate. Corneal scraping was performed, and intensive therapy commenced with fortified cephalothin, gentamicin and polyhexamethylene biguanide; the latter for suspected acanthamoeba, later ruled out by confocal microscopy. Pseudomonas aeruginosa, sensitive to cefataxime, ciprofloxacin and gentamicin, and resistant to chloramphenicol, was isolated. Oral ciprofloxacin, topical ofloxacin and tobramycin were added to the regimen. The corneal infiltrate slowly resolved, leaving a central stromal scar and a slow-to-heal corneal ulcer. Two months later, her left vision was reduced to 6/36. In the first clinical case, chloramphenicol was obtained without medical consultation, and in the second case, advised by an optometrist. Notably both patients had symptoms of reduced vision and pain, as well as significant prior history, that is, rheumatoid arthritis-related dry eye and orthokeratology contact lens wear, placing them at risk of microbial keratitis.1 Ophthalmic preparations of chloramphenicol have been available in Australia as a Schedule 3 medication since 2010, making them widely accessible to the public following consultation with a pharmacist.2 The rationale behind the rescheduling of ophthalmic chloramphenicol was to reduce delays in treatment initiation and patient presentations to general practices and emergency departments.2 Benefit of improved patient access was argued to outweigh the risks of the projected small percentage of misdiagnosis and inappropriate use. Over-the-counter availability of chloramphenicol has observed substantial increases in its use within Australia, New Zealand and the UK following rescheduling in 2010, 2009 and 2005, respectively.3,4 Although clinical guidelines exist, directing the judicious use of chloramphenicol by general practitioners, nurse practitioners, optometrists and pharmacists; the incidence of inappropriate use, bacterial resistance and misdiagnosis is evidently greater than just theoretical. As ophthalmologists, we play a crucial role in educating other health practitioners and in advocating appropriate use of the limited number of topical antibiotics that are at our disposal.
1. Tran T, Samarawickrama C, Petsoglou C, Watson S. Recent cluster of childhood microbial keratitis due to orthokeratology. Clin Experiment Ophthal 2014; 42: 793–4. 2. National Drugs and Poisons Schedule Committee. Record of Reasons, 57th Meeting, 20–21 October 2009.
© 2014 Royal Australian and New Zealand College of Ophthalmologists
Copyright of Clinical & Experimental Ophthalmology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Letters to the Editor
191
Rare case of Langerhans cell histiocytosis of the conjunctiva Langerhans cells are specific antigen presenting cells normally found in the basal layer of the epidermis, squamous epithelia, hair follicles and apocrine/sebaceous glands. Langerhans cell histiocytosis (LCH) is a rare condition characterized by neoplastic proliferation of Langerhans cells and usually affects men and children.1 Langerhans cell histiocytosis lesions are usually unifocal and typically involve the skull, pelvis and femur. Ocular or orbital involvement is very rare. We present a case of rare ocular LCH in a 22-year-old male with conjunctival Langerhans cell histiocytosis. The patient initially presented with a year-long history of temporal injection and irritation of the right eye. He reported no visual deterioration and had previously tried multiple types of eye lubricants without any improvement in symptoms. The patient had recently immigrated to Australia from Afghanistan and reported no other ophthalmic history, medical history or surgical history. He took no regular medications and had no allergies. He reported feeling otherwise well with no other systemic symptoms. On initial examination, visual acuity was 6/5 in both eyes with normal intraocular pressures and no sign of proptosis. Anterior segment examination showed injected temporal conjunctiva and sclera in the right eye. There was no blanching of the injection with 10% topical phenylephrine and no associated fluorescein uptake. Otherwise the eyes were quiet, with clear corneas and no sign of intraocular inflammation. Posterior segment examination was unremarkable bilaterally. The patient was initially treated with topical corticosteroids and oral ibuprofen with a working diagnosis of early nodular non-necrotizing scleritis. Extensive blood testing involving full blood count, electrolytes, liver function tests, inflammatory markers, rheumatoid factor, ANA, C-ANCA/P-ANCA, syphilis, QuantiFERON Gold (Cellestis, Ltd. Carnegie, Australia) and ACE were all normal. Chest X-ray was also normal. Over the next month, the patient’s irritation and right eye injection only settled slightly. Further examination revealed a raised 3 mm diameter fibrovascular mass with feeder vessels within the now reduced right temporal injection (Fig. 1). The mass appeared to be within the conjunctiva with no evidence of any deeper invasion. Excisional biopsy was recommended and undertaken 3 weeks later. The conjunctival lesion was easily excised with adequate macroscopic margins, and cryotherapy was applied to the edges of the conjunctival defect. Histopathology showed aggregates of Langerhans cells with a surrounding infiltrate of eosinophils and lymphocytes. Immunohistochemistry confirmed positive staining for CD1a and S100. Features were consistent with conjunctival LCH. Margins of the specimen were clear.
Competing/conflicts of interest: No stated conflict of interest. Funding sources: No stated funding sources.
Figure 1. Anterior segment photography of the conjunctival Langerhans cell histiocytosis lesion prior to excision. The patient’s eye settled postoperatively with no further irritation or injection and was subsequently referred to haematology. A detailed systemic examination including Computed Tomography (CT) head, chest, abdomen and pelvis, positron emission tomography scan, magnetic resonance imaging head and bone marrow aspirate and trephine showed no evidence of systemic LCH, and a final diagnosis of unifocal conjunctival LCH was made. Conjunctival LCH has only been reported six times previously, three times at the conjunctival limbus,1–3 twice at the caruncle4,5 and once on the epibulbar conjunctiva.6 Although this is a very rare condition, LCH should be recognized as a possible malignant cause of a conjunctival lesion with potentially serious systemic manifestations, and thus should be considered in the differential diagnosis of any conjunctival lesion.
Thomas P Moloney MBBS, Mohammad Ghaznawi MBBS, Sean Cheng MBBS and Andrew Apel FRANZCO Department of Ophthalmology, Princess Alexandra Hospital, Brisbane, Queensland, Australia Received 23 June 2014; accepted 25 June 2014.
REFERENCES 1. Saxena T, Kumar K, Sen S, Tandon R. Langerhans cell histiocytosis presenting as a limbal nodule in an adult patient. Am J Ophthalmol 2004; 138: 508–10. 2. Bakhshi S, Nongpiur ME. Recurrence of Unifocal Limbal Langerhans cell histiocytosis with partial response to chemotherapy and prolonged remission. Pediatr Blood Cancer 2011; 56: 687–8. 3. Kanavi MR, Javadi F, Javadi M, Faramarzi A. Unifocal Langerhans cell histiocytosis stimulating a limbal papilloma. J Ophthalmic Vis Res 2012; 7: 240–3. 4. Stanowsky A, Krey HF, Wagner T. Histiocytosis X (eosinophiles Granulom) der Karunkel. Klin Mbl Augenheilk 1991; 199: 359–61.
© 2014 Royal Australian and New Zealand College of Ophthalmologists
bs_bs_banner
192
Letters to the Editor
5. Kiratli H, Kocabeyoglu S, Saglam A, Soylemezoglu F. Langerhans cell histiocytosis of the caruncle. Clin Experiment Ophthalmol 2007; 35: 661–3. 6. Melamud A, Efrat M, Sova Y, Hod Y, Geyer O. Epibulbar nodule as a symptom of Langerhans cellhistiocytosis. Arch Ophthalmol 2002; 120: 1400–1.
Inappropriate use of topical chloramphenicol results in vision loss Microbial keratitis is an ophthalmic emergency. We present two cases of microbial keratitis in which inappropriate chloramphenicol use delayed diagnosis and hence prompt management. For one patient, the result was loss of the eye, and for the other, a child, lifelong reduced vision from corneal scarring. The first case was a 69-year-old woman who presented with a three-week history of a red, watery right eye and gradually reducing vision. She had been self-treating with chloramphenicol drops and ointment, obtained from her pharmacist without medical consultation. Her history was of keratoconjunctivitis sicca secondary to rheumatoid arthritis, treated with lubricants and topical chloramphenicol when required for ‘red eye’. Her presenting vision was reduced to 6/45 in the affected eye. She had a right corneal abscess, with a slow leak from a corneal perforation. Following corneal scraping, glue was applied and intensive topical fortified cephalothin and gentamicin commenced. Streptococcus pneumonia was isolated from the corneal scrape. Over 48 h, her vision decreased to light perception, and anterior chamber activity increased. Gentamicin was ceased and topical ofloxacin commenced. The organism was then reported to be multi-resistant, and oral ciprofloxacin was commenced. However, despite appropriate management, the infection progressed to involve the vitreous and then sclera, and the eye required evisceration at day five. In the second case, a 16-year-old girl orthokeratology contact lens wearer presented with a two-week history of cloudy left vision and nine days of eye pain. She had worn night-time orthokeratology lenses for three years. At the onset of her symptoms, her optometrist had advised her to use chloramphenicol eye drops. After one week of worsening symptoms, she sought treatment from an ophthalmologist. Her presenting vision was 6/15 in the affected eye. She had a central corneal infiltrate with a surrounding ring
Nikki McDerby MNedSci MPharm,1 Stephanie L Watson PhD FRANZCO,2,3 Dana Robaei PhD FRANZCO2,4 and Mark Naunton BPharm(Hons) PhD1 1 Faculty of Health, Discipline of Pharmacy, University of Canberra, Canberra, Australian Capital Territory, 2Save Sight Institute, University of Sydney, 3Sydney Eye Hospital, and 4Department of Ophthalmology, Westmead Hospital, Sydney, New South Wales, Australia Received 19 October 2014; accepted 26 October 2014.
REFERENCES
Competing/conflicts of interest: No stated conflict of interest. Funding sources: Dr Robaei is funded by an NHMRC Early Career Fellowship (APP1073846). Professor Watson is funded by an NHMRC Career Development Fellowship (APP1050524). The two cases are part of the Serious Ocular Infections Project (SOIP), funded by the Sydney Eye Hospital Foundation.
infiltrate. Corneal scraping was performed, and intensive therapy commenced with fortified cephalothin, gentamicin and polyhexamethylene biguanide; the latter for suspected acanthamoeba, later ruled out by confocal microscopy. Pseudomonas aeruginosa, sensitive to cefataxime, ciprofloxacin and gentamicin, and resistant to chloramphenicol, was isolated. Oral ciprofloxacin, topical ofloxacin and tobramycin were added to the regimen. The corneal infiltrate slowly resolved, leaving a central stromal scar and a slow-to-heal corneal ulcer. Two months later, her left vision was reduced to 6/36. In the first clinical case, chloramphenicol was obtained without medical consultation, and in the second case, advised by an optometrist. Notably both patients had symptoms of reduced vision and pain, as well as significant prior history, that is, rheumatoid arthritis-related dry eye and orthokeratology contact lens wear, placing them at risk of microbial keratitis.1 Ophthalmic preparations of chloramphenicol have been available in Australia as a Schedule 3 medication since 2010, making them widely accessible to the public following consultation with a pharmacist.2 The rationale behind the rescheduling of ophthalmic chloramphenicol was to reduce delays in treatment initiation and patient presentations to general practices and emergency departments.2 Benefit of improved patient access was argued to outweigh the risks of the projected small percentage of misdiagnosis and inappropriate use. Over-the-counter availability of chloramphenicol has observed substantial increases in its use within Australia, New Zealand and the UK following rescheduling in 2010, 2009 and 2005, respectively.3,4 Although clinical guidelines exist, directing the judicious use of chloramphenicol by general practitioners, nurse practitioners, optometrists and pharmacists; the incidence of inappropriate use, bacterial resistance and misdiagnosis is evidently greater than just theoretical. As ophthalmologists, we play a crucial role in educating other health practitioners and in advocating appropriate use of the limited number of topical antibiotics that are at our disposal.
1. Tran T, Samarawickrama C, Petsoglou C, Watson S. Recent cluster of childhood microbial keratitis due to orthokeratology. Clin Experiment Ophthal 2014; 42: 793–4. 2. National Drugs and Poisons Schedule Committee. Record of Reasons, 57th Meeting, 20–21 October 2009.
© 2014 Royal Australian and New Zealand College of Ophthalmologists
Copyright of Clinical & Experimental Ophthalmology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
