Linear IgA disease

Purpose: A case of linear IgA disease is reported to alert ophthalmologists and physicians to this unusual cause of chronic cicatrizing conjunctivitis. Methods: Clinical records of a patient suffering from linear IgA disease were reviewed. Results: A 65-year-old woman with a complicated medical history experienced rapidly progressive chronic cicatrizing conjunctivitis leading to corneal perforation. Undiagnosed gingivitis and palatal ulceration had been present for 5 years prior to the onset of ocular symptoms and vitamin C deficiency had followed the consequent dietary restrictions. A diagnosis of linear IgA disease was made on conjunctival biopsy, which demonstrated linear deposits of IgA along the epithelial basement membrane. The perforation was managed successfully with a conjunctival pediculate flap. Control of the inflammation was achieved with systemic prednisolone and cyclophosphamide but at the expense of serious systemic side-effects. Conclusions: Linear IgA disease causes progressive conjunctival cicatrization in many affected individuals. Although dapsone generally controls the inflammation, heavier systemic immunosuppression was required in this case. Involvement of skin or other mucosal surfaces may become symptomatic before the conjunctivitis, and physicians must be educated to refer patients for ophthalmological review on diagnosis. Conversely, ophthalmologists encountering ocular linear IgA disease should be aware of the possibility of other mucosal involvement requiring physician intervention.     

Linear IgA disease

PURPOSE: A case of linear IgA disease is reported to alert ophthalmologists and physicians to this unusual cause of chronic cicatrizing conjunctivitis. METHODS: Clinical records of a patient suffering from linear IgA disease were reviewed. RESULTS: A 65-year-old woman with a complicated medical history experienced rapidly progressive chronic cicatrizing conjunctivitis leading to corneal perforation. Undiagnosed gingivitis and palatal ulceration had been present for 5 years prior to the onset of ocular symptoms and vitamin C deficiency had followed the consequent dietary restrictions. A diagnosis of linear IgA disease was made on conjunctival biopsy, which demonstrated linear deposits of IgA along the epithelial basement membrane. The perforation was managed successfully with a conjunctival pediculate flap. Control of the inflammation was achieved with systemic prednisolone and cyclophosphamide but at the expense of serious systemic side-effects. CONCLUSIONS: Linear IgA disease causes progressive conjunctival cicatrization in many affected individuals.Although dapsone generally controls the inflammation, heavier systemic immunosuppression was required in this case. Involvement of skin or other mucosal surfaces may become symptomatic before the conjunctivitis, and physicians must be educated to refer patients for ophthalmological review on diagnosis. Conversely, ophthalmologists encountering ocular linear IgA disease should be aware of the possibility of other mucosal involvement requiring physician intervention.     

Massive intraocular invasion of conjunctiva by squamous cell carcinoma--a case report

BACKGROUND: Squamous cell carcinoma of the conjunctiva is a mostly solitary growing tumor located at the limbus region, which seldom invades the eye. We present an unusual case of conjunctival carcinoma with marked intraocular involvement, which appeared primarily as a chronic inflammation with corneal ulcer. CASE REPORT: A 89-year-old female patient was referred to our institution with a suspected ulcus rodens of the cornea. Since one year a refractory conjunctivitis persisted despite of different local treatment. At first presentation, a marked bacterial keratoconjunctivitis with circular necrosis of the conjunctiva, corneal infiltration and perforation was seen. Visual acuity of the right eye was light perception. The left eye was unremarkable. Enucleation of the right eye was performed. Histopathologic examination revealed the diagnosis of squamous cell carcinoma of the conjunctiva with intraocular involvement. There was a marked infiltration of the choroid even behind the equator. No metastases were found. CONCLUSION: Advanced squamous cell carcinoma associated with superinfection may present as a primary inflammatory process. There may be extensive involvement of the choroid. Metastases are probably rare even in advanced cases of this type of carcinoma.     

Behçet's disease: corneal perforation as an ocular manifestation

Purpose Although ocular manifestations are common among patients with Behçet's disease, corneal perforation has not been reported in the literature. We report an unusual case of Behçet's disease with corneal perforation as the main ocular involvement.
Methods/results An elderly Chinese patient was referred for eye examination because of clinical suspicion of Behçet's disease. Eye examination showed evidence of long-standing autoimmune disease of the eye, mild iritis and a corneal perforation in the left eye. Despite evidence of chronicity and corneal perforation, the patient was asymptomatic. The perforation was successfully treated with cyanoacrylate glue.
Conclusions While anterior segment involvement is common in Behçet's disease, this case highlights an unusual and hitherto unreported ocular involvement.     

Gonococcal conjunctivitis complicated by perforating corneal abscess in an adult

We present a case of bilateral purulent conjunctivitis complicated by ocular perforation of the right eye secondary to fulminant corneal melt in a 29-year-old man. He developed urethritis after a sexual contact with a prostitute 3 weeks previously. Microbiological analyses of conjunctival and urinary cultures were positive for Neisseria gonorrhoeae resistant to penicillins, tetracyclines, and fluoroquinolones. Progression was favorable with a 15-day course of high doses of parenterally administered antibiotics associating imipenem and fosfomycin. Keratoplasty was done after 3 months. This observation is a good example of the problems raised by gonococcal conjunctivitis in adults. Extremely rare in developed countries, it remains widely unrecognized by ophthalmologists. It is a sexually transmitted disease usually resulting from autoinoculation from an infected genital site. The risk of marginal purulent corneal melt, which can lead to fulminant perforation, warrants prompt microbiological analysis and early parenteral antibiotic treatment.     

Bilateral spontaneous corneal perforation associated with complete external ophthalmoplegia in mitochondrial myopathy (kearns-sayre syndrome)

PURPOSE: Mutations of mitochondrial DNA can lead to a variety of pheno- and genotypically heterogeneous diseases. Kearns-Sayre syndrome is caused by the deletion of several mitochondrial genes. The syndrome is characterized by chronic progressive external ophthalmoplegia, tapetoretinal degeneration, and severe generalized myopathy. CASE REPORT: We report on a 36-year-old female patient with Kearns-Sayre syndrome, confirmed by biochemistry, histology, and genetics. Over a period of 10 years, progressive ophthalmoplegia led to recurrent conjunctivitis, keratitis, and corneal ulceration. Almost total external ophthalmoplegia including involvement of the orbicularis oculi muscles was observed. Despite advanced ptosis, there was lagophthalmos of 2 mm with near complete extinction of globe motility in both eyes. The left eye showed a peripheral corneal perforation parallel to the lower limbus. After successful penetrating keratoplasty in the left eye, despite preventive measures, a peripheral corneal perforation also occurred in the right eye. Penetrating keratoplasty was therefore also performed on the right eye. To achieve a satisfactory functional result, large-diameter transplants were necessary in both eyes. To prevent immune reactions, cyclosporine therapy was initiated prophylactically. Sixteen and 9 months after penetrating keratoplasty, the corrected visual acuity was 20/60 in the right eye and 20/100 in the left eye, with clear transplants on both sides. DISCUSSION: Patients with progressive ophthalmoplegia require thorough neurologic investigation and evaluation. Lagophthalmos in the presence of almost absent globe motility requires extensive preventive measures to avoid exposure keratitis. In spite of this, in the presented case, corneal perforation of the second eye could not be prevented.     

Chronic dacryoadenitis misdiagnosed as eyelid edema and allergic conjunctivitis.

PURPOSE: To report the case of a 53-year-old woman with a 2-year history of episodic upper eyelid swelling and nonspecific complaints, who was diagnosed as having allergic conjunctivitis. METHODS: A complete ocular examination, orbital computerized tomographic (CT) scans followed by complete physical and systemic examinations. RESULTS: The results of physical and systemic examinations were unremarkable for systemic lymphoma and a primary focus of cancer. The results of the ocular examination were normal. CT scans demonstrated well-defined lesions bilaterally with a homogeneous internal structure in the lacrimal gland fossa, which suggested a diagnosis of chronic dacryoadenitis. The differential diagnosis included lymphoma and orbital metastases. The patient refused a biopsy and was started on a tapering dose of 60 mg oral prednisolone daily. The follow-up CT scans 1 month after cessation of 6-week oral corticosteroid treatment showed near complete resolution of the orbital lesions. CONCLUSION: This case demonstrates that orbital inflammation can be misdiagnosed as refractory allergic conjunctivitis.     

Primary meningococcal conjunctivitis in an adult

PURPOSE: To describe a case of primary meningococcal conjunctivitis mimicking epidemic keratoconjunctivitis. METHODS: Review of a case history and current literature. RESULTS: A 28-year-old man presented with 5 days of a bilateral follicular conjunctivitis and subepithelial corneal infiltrates. Initial diagnosis of adenoviral conjunctivitis was made. Initial Gram stain of conjunctival exudates was negative. Cultures grew Neisseria meningitidis. Systemic antibiotics were instituted with adjunctive topical therapy. The conjunctivitis resolved with no evidence of systemic invasion. CONCLUSIONS: The clinical picture of Neisseria meningitidis conjunctivitis can overlap with other infectious organisms including adenovirus. Early diagnosis and systemic antibiotic therapy are critical to prevent bacteremic spread of infection.     

Indolent corneal ulcers in a patient with congenital insensitivity to pain with anhidrosis: a case report and literature review

PURPOSE: To report a case of bilateral corneal neurotrophic ulcer in patient with congenital insensitivity to pain with anhidrosis (CIPA) and review the literature. CASE REPORT: A 6 year-old boy presented with bilateral central corneal sterile ulcer, decreased corneal sensitivity, moderately altered corneal reflex and normal tearing response. History taken, systemic evaluation and medical chart review were undertaken. DISCUSSION: Fifty-two cases of CIPA have been reported worldwide. Fourteen cases had corneal involvement. The clinical picture of our patient is characteristic of CIPA. CONCLUSIONS: Congenital insensitivity to pain with anhidrosis may present as neurotrophic corneal ulcer. We report herewith, this vision threatening corneal congenital abnormality. Early diagnosis and prompt treatment are mandatory to prevent corneal complications such as scarring and perforation.     

Lymphogranuloma venereum conjunctivitis with a marginal corneal perforation

The authors have recently treated a case of Parinaud's oculoglandular syndrome due to Chlamydia trachomatis serotype L2, a causative agent of lymphogranuloma venereum (LGV). The ocular manifestations included a mixed papillary-follicular conjunctivitis with fleshy superior limbal lesions in both eyes. A superior marginal corneal perforation requiring a therapeutic corneal graft was present in the right eye. The patient had vaginitis, inguinal lymphadenopathy, a history of Sj?gren's syndrome, and seropositivity to human immunodeficiency virus (HIV). The ocular disease resolved completely after 6 weeks of oral tetracycline therapy.     

Corneal complications following abuse of topical anesthetics

PURPOSE: To describe corneal complications in three patients following abuse of topical anesthetics. MATERIALS AND METHODS: We describe one case with bilateral corneal perforation, another with unilateral corneal perforation and a third case of chronic keratitis following excessive use of topical anesthetics. RESULTS: Two patients continued to instill topical anesthetic drops despite all recommendations to stop. The result was a bilateral corneal perforation in the first case and a large unilateral descemetocele in the second. The third patient who had chronic toxic keratitis discontinued the anesthetic drops and after the appropriate treatment the cornea returned to normal. Corneal grafting and conjunctival flaps were used to seal the corneal perforation but the long-term anatomical and functional results were very poor. CONCLUSIONS: The initial presentation of this rare clinical entity creates difficulties in reaching a correct diagnosis. A presumed acanthamoeba keratitis is the first choice among many similar conditions. Thus abuse of topical ocular anesthetic drops should be included in the differential diagnosis of cases of chronic keratitis as it may masquerade as acanthamoeba keratitis. A current or past history of psychiatric and mental disorders or psychoactive substance abuse is important in the diagnosis. Functional and anatomical results after appropriate treatment are usually poor. Psychiatric counselling is extremely helpful and is in fact mandatory in the management of these patients.     

Severe Aspergillus keratomycosis treated with itraconazole per os

A case of deep traumatic keratomycosis due to Aspergillus fumigatus with anterior chamber involvement is reported. Corneal perforation was threatening because of the large deep and long standing ulcer. This case emphasizes the difficulties of etiological diagnosis and treatment of keratomycosis. The authors analyse the peculiarities of corneal mycotic abcess and emphasize the importance of corneal cultures; they discuss the most recent therapeutic protocols for these lesions. After a very poor response to conventional antifungal therapy, total and quick recovery was acquired using itraconazole per os and topical Amphotericine B. The efficiency of itraconazole proves its antifungal activity against Aspergillus fumigatus and its good penetration to the deeper layers of the cornea and of the anterior chamber.     

Corneal perforation due to limbal involvement in Sézary syndrome

Background  

The majority of lymphomas involving the eye and ocular adnexa are B-cell lymphomas. Ocular involvement by T-cell lymphoma is rare. We report a case of corneal perforation due to direct involvement of the corneal limbus by lymphoma in a patient with Sézary syndrome.     

PCR扩增技术在新生儿结膜炎中的诊断应用

目的 探讨PCR扩增技术在新生儿结膜炎临床诊断中的价值。方法 采用PCR扩增技术和直接涂片镜检两种方法，分别检测1997年9月～2002年10月到中山大学中山眼科中心就诊的200例新生儿结膜炎息儿的结膜标本中的淋球菌、沙眼衣原体和解脲支原体。结果 PCR扩增检测出18例淋球菌阳性(9％)，65例沙眼衣原体阳性(32．5％)，8例解脲支原体阳性(4％)；直接涂片镜检法检出14例淋球菌阳性(7％)，47例沙眼衣原体阳性(23．5％)，5例解脲支原体阳性(2．5％)。PCR扩增的阳性率比直接涂片镜检高。结论 PCR扩增技术是一种快速、简便、灵敏的检测新生儿结膜炎常见致病病原体的方法，与传统直接涂片镜检法比较，具有一定的优势。     

Ocular complications associated with brucellosis in an endemic area

PURPOSE: To report the ocular manifestations associated with brucellosis in an endemic area. METHODS: We prospectively evaluated 147 patients with the diagnosis of brucellosis between May 1996 to May 2000 and recorded the ocular and systemic findings. The diagnosis was based on clinical findings, positive serological and bacteriological tests (Brucella agglutination test: over 1/160 titer, blood culture). RESULTS: Thirty-eight patients (26.0%) with brucellosis had ocular complications: conjunctivitis in 26 (17.7%), anterior uveitis in six (4.1%), posterior uveitis in one (0.7%), dacryoadenitis in two (1.4%), episcleritis in three (2.1%). Three of the seven patients with uveitis had spondylitis associated with brucellosis. Osteoarticular complications in brucellosis were more frequent in the patients with ocular involvement though the difference was not statistically significant compared with patients without ocular involvement. CONCLUSIONS: Ocular manifestations are frequent in brucellosis so an ophthalmologic examination should be routinely performed in patients with brucellosis in endemic areas.     

Acute lacrimal gland swelling with intracranial extension and without any neurological features – A rare case report

We report a case of young adult female with swelling in the superolateral aspect of left orbit for two weeks which was diagnosed as tubercular dacryoadenitis with intracranial extension without any neurological features. Tubercular dacryoadenitis is very rare but still makes an important differential diagnosis of lacrimal gland swellings especially in endemic areas like India. Few cases of tubercular dacryoadenitis have been reported in the past; but not a single case with intracranial extension in young adults with short history and without neurological symptoms. Although radiological investigations are routinely done in orbital lesions, it is advisable do so even in acute cases to look for intracranial extension before the appearance of neurological features. Tubercular dacryoadenitis though rare, should be kept as a differential diagnosis of acute or chronic lacrimal gland swellings in endemic areas.     

The giant fornix syndrome: an unrecognized cause of chronic, relapsing, grossly purulent conjunctivitis

AIM: To describe a group of elderly patients presenting with chronic, relapsing, copiously purulent conjunctivitis, in which the condition was often perpetuated by the sequestration of a large number of bacteria on a protein coagulum lodged in the recesses of a large upper conjunctival fornix. PATIENTS AND METHODS: Retrospective review of a noncomparative case series, drawn from patients attending the lacrimal clinic at Moorfields Eye Hospital. OUTCOME MEASURES: Characterization of this unrecognized syndrome and its response to treatment. RESULTS: Twelve patients (10 female) presented between the ages of 77 and 93 years (mean, 85; median, 86) with a history of chronic relapsing bacterial conjunctivitis affecting, with 2 exceptions, just one eye. All had experienced multiple episodes of markedly purulent conjunctivitis and chronic ocular discharge for between 8 and 48 months (mean, 23.5; median, 24) before referral, and the patients had received multiple courses of treatment. Three had successful external dacryocystorhinostomy (for nasolacrimal duct occlusion) before the final diagnosis of giant fornix syndrome was made, 9 had developed corneal vascularization and scarring before referral, and 5 had suffered prior spontaneous corneal perforation or thinning. All patients had deep upper conjunctival fornices in association with the changes of age-related dehiscence of the levator muscle aponeurosis. Copious amounts of thick, purulent debris and a yellow coagulum were lodged in the depths of the upper fornix-this debris universally culturing Staphylococcus aureus. The condition settled rapidly on appropriate systemic antibiotics (ciprofloxacin or ofloxacin), intensive topical antibiotics, and high-dose, high-potency steroids; some patients required repeated treatment or needed to continue the use of a single drop of a combined steroid-antibiotic to prevent relapse. CONCLUSION: The capacious upper fornix of the elderly may harbor a coagulum colonized by S. aureus, leading to chronic conjunctivitis that may lead to severe sight impairment due to toxic keratopathy and secondary corneal vascularization.     

Ocular involvement in acute febrile neutrophilic dermatosis (Sweet syndrome): new cases and review of the literature

Sweet syndrome (acute febrile neutrophilic dermatosis) is a dermatologic disorder with accompanying features of systemic inflammation. It is commonly associated with conjunctivitis, but a variety of types of ocular inflammation have been reported. The ocular manifestations of Sweet syndrome include periorbital and orbital inflammation, dacryoadenitis, conjunctivitis, episcleritis, scleritis, limbal nodules, peripheral ulcerative keratitis, iritis, glaucoma, and choroiditis. The ocular inflammation appears concurrently with skin lesions. An overview of Sweet syndrome is presented with a review of cases in the literature describing ocular involvement. We report two additional cases of ocular involvement, one with conjunctivitis and a second with iritis, peripheral ulcerative keratitis, and episcleritis. Of the 20 cases, half were bilateral. Thirteen cases occurred in the setting of classical or idiopathic Sweet syndrome and seven in association with malignancy. Biopsies of ocular tissue were infrequent, but, in the seven cases where ocular tissue was analyzed, the histopathology was similar to that of the cutaneous lesions. The ocular complications of Sweet syndrome resolved with systemic administration of corticosteroid or cyclosporine. Topical ocular steroid treatment was frequently used in conjunction with oral steroid but may not have been valuable.     

Enfermedad orbitaria relacionada con Ig-G4

Case report

The case is presented of a 64-year-old woman with bilateral palpebral swelling and dacryoadenitis, exophthalmos, and a history of chronic rhinitis and asthma. An increase in serum IgG4 was observed, and an incisional biopsy of lacrimal glands was performed, which showed fibrosis and a lymphoplasmacytic infiltrate with IgG4 producing cells.