Interstitial keratitis presenting as the ocular manifestation of undiagnosed hidradenitis suppurativa.

Interstitial keratitis is defined as nonulcerative inflammation of the corneal stroma resulting from a bacterial or viral infection, parasitic antigen, or autoimmune response. Hidradenitis suppurativa is an inflammatory skin condition that presents with painful and inflamed boils in apocrine gland-bearing regions of the body, typically with onset around puberty, and ocular comorbidities exist in only a small percentage of these patients. Herein, we describe a unique ocular presentation of hidradenitis suppurativa including pronounced bilateral interstitial keratitis and association with high titer c-ANCA (antineutrophil cytoplasmic antibodies).

Bilateral interstitial keratitis following COVID-19: a case report.

BACKGROUND: Although the primary target of severe acute respiratory syndrome coronavirus 2 is the respiratory tract, the expression of the angiotensin-converting enzyme 2 receptor in other tissues facilitates viral entry in others parts of the body, including ocular structures. Ocular manifestations may occur before, during, or after systemic infection. CASE PRESENTATION: We report the case of a 60-year-old male who presented with bilateral interstitial keratitis after the onset of COVID-19, with ocular symptoms starting within 7 days after systemic symptoms. Laboratory investigation did not identify any alternative etiology for his disease, although the possibility of Epstein-Barr virus or herpes simpex virus could not be definitively ruled out. The patient had already developed significant corneal scarring and visual debilitation by the time topical steroids were initiated, and his final corrected visual acuity with rigid gas permeable contact lenses was 20/50 and 20/80 in the right and left eye, respectively. CONCLUSIONS: The involvement of ocular tissue by the virus can lead to permanent sequelae such as severe visual loss, and clinicians should be aware of and recognize ophthalmic manifestations of this disease to prompt early intervention.

Three-dimensional in vivo evaluation of the cornea in patients with unilateral posterior interstitial keratitis.

Purpose: The purpose of this study was to investigate the in vivo morphologic features of the cornea in patients with unilateral posterior interstitial keratitis. Methods: Seven eyes of 7 patients with unilateral posterior interstitial keratitis were examined by slit-lamp biomicroscopy, anterior segment optical coherence tomography (AS-OCT), and in vivo confocal microscopy (IVCM). The imaging features of the cornea were evaluated and analyzed. Results: By slit-lamp examination, the posterior corneal stromal opacities were observed in all 7 eyes, and deep neovascularization in 4 eyes. The posterior stromal opacities showed higher reflectivity with an intact overlying epithelium by AS-OCT and did not invade the Bowman's layer in all cases. IVCM revealed highly reflective dispersed microdots, needle-shaped bodies, and increased reflectivity of keratocytes in the lesion site in all patients. Active Langerhans cells and an attenuated subbasal nerve plexus were observed in 5 eyes. After treatment, the active Langerhans cells disappeared; however, highly reflective microdots and needle-shaped bodies remained. Conclusion: The three-dimensional evaluation of slit-lamp biomicroscopy, AS-OCT, and IVCM may help in the early diagnosis of patients with posterior interstitial keratitis.

An Unusual Presentation of Cogan's Syndrome With a Renal Tumor: A Report of a Rare Case.

Cogan's syndrome (CS) is a rare disorder of an unknown origin characterized by inflammatory eye disease and vestibuloauditory symptoms, primarily affecting young white adults, without a hereditary pattern. The exclusion of other diseases makes diagnosis difficult, and it is likely underreported in the literature. A 74-year-old previously healthy African American male presented with ear and jaw pain, later accompanied by vestibular symptoms, fever of unknown origin, aortitis, and a third-degree heart block. The workup revealed incidental renal cell carcinoma and interstitial keratitis. This case highlights the challenge of diagnosing an atypical presentation of CS with late-onset interstitial keratitis by excluding other complex syndromes.

Cogan's syndrome is more than just keratitis: a case-based literature review.

BACKGROUND: Cogan's syndrome (CS) is a rare autoimmune disorder characterized by non-syphilitic interstitial keratitis (IK) and Menière-like cochlear vestibular symptoms, which may also have systemic effects. Corticosteroids are first-line treatment. DMARDs and biologics have been used to treat ocular and systemic symptoms of CS. CASE PRESENTATION: This is a case of a 35-year-old female who reported hearing loss, eye redness and photophobia. Her condition progressed to a sudden sensorineural hearing loss, tinnitus, and constant vertigo accompanied by cephalea. CS was diagnosed after excluding other diseases. The patient still developed bilateral sensorineural hearing loss after receiving hormone, methotrexate, cyclophosphamide, and a variety of biological agents. Joint symptoms were relieved after treatment with a JAK inhibitor (tofacitinib), and hearing did not deteriorate further. CONCLUSIONS: CS should be involved in the differential diagnosis of keratitis. Early identification and intervention of this autoimmune disease can minimize disability and irreversible damage.

A case report of interstitial keratitis and secondary glaucoma after cataract surgery that may be related to late congenital syphilis.

BACKGROUND: The destruction of blood eye barrier and the administration of corticosteroid eyedrops after phacoemulsification surgery can lead to the replication of the local potential pathogens. With the rapid increase and popularization of cataract surgery, all kinds of rare postoperative complications have appeared. Here, we report a case of interstitial keratitis and secondary glaucoma after cataract surgery, which may be related to late congenital syphilis, which eventually led to blindness in the right eye. We hope that the timely report of this case will enable doctors to pay more attention to the possibility of potential pathogen replication after cataract surgery, and enable more patients to receive reasonable and effective treatment. CASE PRESENTATION: A 63-year-old female was referred to our clinic for investigation with a 1-week history of moderate pain in the right eye and ipsilateral headache in January 2020. She had cataract surgery on her right eye two years ago and on her left eye one year ago. The intraocular pressure (IOP) in the right eye was 43.2 mmHg and that in the left eye was 28.5 mmHg. Her right eye underwent medication, trabeculectomy and finally was subjected to ciliary body photocoagulation to control the IOP. The IOP of the left eye was well controlled by regular use of eye drops. In addition to the elevated IOP, the inflammation of the anterior segment and corneal stroma was found. Before cataract surgery, bilateral corneal opacities was revealed, but after cataract surgery, interstitial keratitis in both eyes was gradually aggravated, during the follow-up period from 2019 to 2021. She informed us that she had suffered from decreased vision in both eyes and was diagnosed with bilateral keratitis and congenital syphilis at the age of 20. In 2018, the serologic test for syphilis was positive in blood (Chemiluminescence analysis (CLIA): + ; Toluidine red unheated serum test (TRUST): + , titer was 1:1). However, four tests for TRUST were negative in 2019 and 2020, so she was not treated for syphilis. CONCLUSION: This case of glaucoma and interstitial keratitis might be secondary to ocular inflammation caused by late congenital syphilis. The ocular inflammation and the activation of syphilis may be related to cataract surgery.

Case report of interstitial keratitis in pregnancy.

Purpose: To report an unusual case of interstitial keratitis and lipid keratopathy in a pregnant woman with unknown etiology and atypical clinical course. Observations: A 15 weeks pregnant 32-year-old female daily soft contact lens wearer presented with 1 month of right eye redness and intermittent blurry vision. Slit lamp examination revealed sectoral interstitial keratitis with stromal neovascularization and opacification. No underlying ocular or systemic etiology was identified. The corneal changes were unresponsive to treatment with topical steroids and progressed over the ensuing months of her pregnancy. On continued follow up, the cornea demonstrated spontaneous partial regression of the opacification in the post-partum period. Conclusions and importance: This case illustrates a possible rare manifestation of pregnancy physiology in the cornea. It also emphasizes the utility of close follow-up and conservative management in pregnant patients with idiopathic interstitial keratitis not only to avoid intervention during pregnancy but also because of the possibility of spontaneous improvement or resolution of the corneal changes.

Ocular Involvement Preceded the Onset of Cutaneous Lesions in Hydroa Vacciniforme-Like Lymphoproliferative Disorder: A Case Report.

PURPOSE: To report a case of ocular involvement associated with hydroa vacciniforme-like lymphoproliferative disorder (HVLPD). CASE REPORT: A 10-year-old HVLPD boy suffered conjunctivitis, interstitial keratitis, and anterior uveitis sequentially during the whole course. Interestingly, this case manifested initially only with ocular findings, which preceded 1 year earlier than the onset of cutaneous lesions. And his later ocular findings occurred simultaneously with cutaneous lesions. The patient was treated with oral prednisone, ganciclovir, and light protection. Topical corticosteroid drops used to control ocular inflammation. Since then, he has not had any flares of ocular inflammation, and the cutaneous lesions improved. Although corneal nebula had been formed, the vision was still good. CONCLUSION: Our case was supportive of ocular involvement in HVLPD. Ophthalmologists should be aware of ocular involvement in HVLPD could be preceded the onset of cutaneous lesions, and prudently perform a careful ophthalmic examination at regular intervals to limit long-term sequelae.

Cogan syndrome masquerading as corneal ectasia.

PURPOSE: To report a case of Cogan syndrome that presented with the appearance of bilateral asymmetric corneal ectasia on Scheimpflug tomography. METHODS: Case Report and Literature Review. RESULTS: A 43-year-old woman previously diagnosed with keratoconjunctivitis sicca and presumed keratoconus presented with seven months of episodic eye pain and progressive bilateral blurry vision with new onset bilateral monocular diplopia. Review of symptoms were significant for tinnitus, vertigo, and sensorineural hearing loss that began many months after her initial presentation for visual symptoms. Scheimpflug tomography showed asymmetric focal steepening on anterior curvature with corresponding focal total corneal thinning, focal posterior elevation, and abnormal ARTMax (205 OD, 103 OS) and BAD-D (2.75 OD, 5.6 OS) values. Clinical examination was notable only for faint anterior corneal stromal inflammation without neovascularization, but there was significant corresponding focal hyperreflectivity on anterior segment optical coherence tomography (OCT) examination with focal epithelial hypertrophy rather than thinning. Given the combined findings of interstitial keratitis and sensorineural hearing loss the patient was diagnosed with Cogan syndrome. She responded well to topical steroids and systemic immunosuppressive therapy, with near resolution of her abnormal topographic and tomographic findings and resolution of monocular diplopia in both eyes. CONCLUSIONS: Cogan syndrome should be suspected for any patient with corneal stromal findings and associated with vertigo and/or hearing loss. Anterior segment optical coherence tomography (OCT) can distinguish between ectatic and inflammatory diseases and may help make the appropriate diagnosis in subtle cases.

Syphilitic interstitial keratitis treated with topical tacrolimus.

PURPOSE: To report a case of syphilitic interstitial keratitis successfully managed with topical tacrolimus after the development of steroid-induced intraocular pressure elevation in a pediatric patient. OBSERVATIONS: A 4-year-old female with a history of congenital syphilis that was reportedly treated after birth presented with bilateral conjunctival redness, tearing, and photosensitivity. Initial ophthalmic examination revealed corneal vascularization with diffuse haze of the right eye and circumferential vascularization with stromal infiltrates of the left eye. She was diagnosed with bilateral syphilitic interstitial keratitis and initially managed with topical steroids but developed steroid-induced elevation of her intraocular pressure. She experienced several recurrences of keratitis as steroids were tapered. After a recurrence in her right eye, she was treated with topical tacrolimus. Since then, she has remained recurrence-free for almost three years with normal intraocular pressure. CONCLUSION AND IMPORTANCE: Tacrolimus represents a novel alternative for the treatment of syphilitic interstitial keratitis, which is particularly useful in patients that develop elevated intraocular pressures in response to long-term treatment with steroid eye drops.

Bilateral retrocorneal hyaline scrolls secondary to asymptomatic congenital syphilis: A case report.

BACKGROUND: Retrocorneal hyaline scrolls are a rare phenomenon. We report a case of bilateral retrocorneal hyaline scrolls that were likely induced by asymptomatic congenital syphilis. CASE SUMMARY: A 71-year-old woman presented with blurred vision due to cataracts. Slit-lamp microscopy revealed bilateral hyaline scrolls with a dichotomous branching pattern extending to the anterior chamber or rods attaching to the rough posterior surface of the cornea. The patient was positive for syphilis-specific antibodies, with no ocular or systemic evidence of congenital or acquired syphilis. Binocular cataract, retrocorneal scroll, and corneal endothelial gutta were considered. The scroll of the right eye was removed during cataract surgery and further observed using hematoxylin-eosin staining and scanning electron microscopy. The cornea of the right eye remained transparent, and the residual scroll seemed stable, however, the corneal endothelial density declined at 13 mo after surgery. In vivo confocal microscopy revealed coalescence of corneal guttae at the level of the corneal endothelium or adhesion to the posterior surface of the endothelium, with enlarged endothelial cells in both eyes. Activated keratocytes in the stroma and a highly reflective acellular structure at the level of the Descemet's membrane were observed. The removed scroll had a cartilage-like hardness and a circularly arranged fiber-like acellular structure. CONCLUSION: Occult congenital syphilis could induce corneal endothelial gutta and the formation of retrocorneal scrolls without other signs of ocular syphilis.

Interstitial Keratitis with Corneal Perforation as the Presenting Sign of Systemic Tuberculosis.

Background: Ocular surface and corneal involvement in tuberculosis is seldom seen. We report a patient of pulmonary and presumed ocular tuberculosis with immune keratitis along with corneal perforation as the presenting signs.Methods: A middle-aged male presented with stromal keratitis, deep corneal vascularization, and two corneal perforations. Necrotic Mantoux test and cavitary lesion on computerized tomography of the chest clinched the diagnosis of tuberculosis-related interstitial keratitis.Results: Tubercular interstitial keratitis was successfully managed with antitubercular therapy, oral and topical steroids while cyanoacrylate glue was used to seal the corneal perforations.Conclusions: Although corneal involvement is uncommon in ocular tuberculosis, in patients with interstitial keratitis who respond poorly to conventional therapy, a possibility of systemic tuberculosis should be considered in endemic countries.

[Interstitial keratitis diagnosis and treatment (French translation of the article)]. / Kératites interstitielles : prise en charge diagnostique et thérapeutiques.

Interstitial keratitis is a non-ulcerative, non-suppurative, more or less vascularized inflammation of the corneal stroma. The corneal lesions result from the host response to bacterial, viral (40% of cases) or parasitic antigens, or from an autoimmune response (1% of cases) without active corneal infection. The natural history of the disease is divided into two phases : acute and cicatricial. This type of keratitis is less common than ulcerative bacterial keratitis, but it is a non-negligible cause of visual loss. It is associated with systemic or infectious disease and requires early diagnosis and appropriate treatment to optimize visual prognosis and avoid other complications.

Interstitial keratitis diagnosis and treatment.

Interstitial keratitis is a non-ulcerative, non-suppurative, more or less vascularized inflammation of the corneal stroma. The corneal lesions result from the host response to bacterial, viral (40% of cases) or parasitic antigens, or from an autoimmune response (1% of cases) without active corneal infection. The natural history of the disease is divided into two phases: acute and cicatricial. This type of keratitis is less common than ulcerative bacterial keratitis, but it is a non-negligible cause of visual loss. It is associated with systemic or infectious disease and requires early diagnosis and appropriate treatment to optimize visual prognosis and avoid other complications.

Presumed Tuberculous Sclerokeratitis Presenting with Hypopyon.

Purpose: To describe a case of presumed tuberculous sclerokeratitis which presented with anterior uveitis and hypopyon Methods: A retrospective chart review Results: A 23-year-old female presented with nodular scleritis, peripheral corneal opacities, and severe anterior chamber reaction with hypopyon. Her Mantoux test and interferon gamma release assay were positive and high-resolution computerized tomography of chest revealed right hilar lymphadenopathy. Aqueous aspirate from anterior chamber paracentesis of her right eye was negative for Mycobacterium tuberculosis genome. She responded to antitubercular treatment and oral corticosteroid Conclusions: Tuberculous sclerokeratitis can rarely present with hypopyon and pose a challenge in diagnosis for the clinicians.

An unusual case of post-cochlear implant performance degradation in a patient with suspected Cogan's syndrome.

Cogan's Syndrome is an autoimmune disorder that can affect the ear, eye and other organs. Although rare, Cogan's Syndrome is particularly relevant to the cochlear implant surgeon because the resulting hearing loss is often bilateral and the majority of cases progresses to profound levels where cochlear implantation may be indicated. There are many issues relating to this condition that concern the cochlear implant surgeon. Its rarity, lack of specific laboratory diagnostic tests and variability in the onset and types of manifestation relating to the ear, eye and other organs often pose diagnostic difficulties. Pre-operatively, the cochlear implant surgeon must anticipate and exclude the possibility of cochlear luminal obliteration and ossification. Although the post-implant hearing results are expected to be generally good in Cogan's Syndrome, the possibility of adverse hearing outcomes cannot be ruled out whether in the initial or subsequent post-operative period. The possible side effects of long-term immuno-suppressive therapy on the well-being of the cochlear implant are to be appreciated and managed. A case which posed much difficulty in management is presented and discussed to highlight some of these challenges.

Sudden sensorineural hearing loss in atypical Cogan's syndrome: A case report.

The Cogan's syndrome (CS) is a very uncommon inflammatory condition that appears in young adults without a gender predisposition. It can be presented as typical, with interstitial non-syphilitic keratitis and Ménière-like audiovestibular manifestations. An atypical form of CS involves uveitis, scleritis, episcleritis, and systemic vascuitis symptoms. This is a case of a 41-year old male who reported eye redness, photophobia, and gait instability. His condition progressed to a sudden sensorineural hearing loss, tinnitus, and constant vertigo accompanied by cephalea. He was evaluated by the Otolaryngology, Ophthalmology, and Neurology departments giving rise to CS, Ménière's syndrome, acoustic neuroma, glaucoma, multiple sclerosis, and meningioma as differential diagnoses. The patient was prescribed with oral and intravenous steroids, ophthalmic antibiotics and steroids, as well as oral omeprazole. The patient was discharged, without his eye manifestations, and for his clinical progress and underwent conventional and speech audiometry, otoacoustic emissions, nystagmography, tympanometry, and auditory steady-state response, that showed a vestibular disfunction and a severe sensorineural hearing loss. His follow-up, six months later resulted with a normal vestibular function and an improvement from severe to mild sensorineural hearing loss. The Cogan's syndrome is a rare condition which can leave the patient with permanent incapacitating secuelae including profound hearing loss. Effective treatment is necessary to avoid complications and improve the patient's condition and life quality.

Keratoconus-like tomographic changes in a case of recurrent interstitial keratitis.

BACKGROUND: The purpose of this report was to describe a single case of recurrent interstitial keratitis in a young patient leading to keratoconus-like corneal changes. RESULTS: Over the 2 years follow-up, the patient developed clinical signs of keratoconus with Vogt's striae, Fleischer Ring and paracentral stromal thinning in the affected eye only. The tomographic measurements revealed a rapid reduction of corneal thickness from 581 to 303 µm and an irregular steepening of the pathological cornea. True net corneal power increased from 42.8 to 48.8 dioptres (D) and maximal power of the anterior surface from 44.9 to 66.9 D. Best-corrected visual acuity (BCVA) decreased from 20/20 to 20/200. A penetrating excimer laser keratoplasty was performed to restore vision and corneal stability. CONCLUSION: Keratoconus-like changes can occur in young patients with recurrent interstitial keratitis and request corneal transplantation.

Human T Cell Leukemia Virus Type 1 and Eye Diseases.

Human T cell leukemia virus type 1, also known as human T lymphotropic virus type 1 (HTLV-1), is a retrovirus that encodes a reverse transcriptase, which translates viral RNA into a DNA provirus that is integrated into the host genome. The virus was found to be a causative agent of adult T cell leukemia/lymphoma (ATL) in the early 1980s, and was also found to cause the neurological disorder tropical spastic paraparesis (TSP)/HTLV-1-associated myelopathy (HAM) and the inflammatory disorder HTLV-1 uveitis in the mid 1980s and early 1990s, respectively. This article reviews eye diseases caused by or related to HTLV-1: HTLV-1 uveitis, ocular and systemic complications of HTLV-1, keratoconjunctivitis sicca, interstitial keratitis, and ATL.

A Case of Acute Interstitial Keratitis in a Patient with Acquired Syphilis

PURPOSE: To report a case of acute interstitial keratitis as the first clinical sign in a patient with latent syphilis. CASE SUMMARY: A 23-year-old female presented with visual impairment and discomfort in her right eye that developed 3 days earlier. The visual acuity in the right eye was 20/200 and corrected to 20/100, and slit lamp examination showed round sub-epithelial opacification in the central cornea with stromal edema and neovascularization on the cornea of the right eye. Whole body tests including serological tests were performed. Under the suspicion of acute interstitial keratitis, topical antibiotics and steroids were applied 4 times a day initially. Serological tests were reactive for venereal disease research laboratory test (VDRL). Under the suspicion of acute interstitial keratitis due to syphilis, fluorescent treponemal antibody absorption test IgM/IgG (FTA-ABS IgM/IgG) was performed; a positive result for FTA-ABS IgG led to diagnosis of acute interstitial keratitis with latent syphilis. During treatment, systemic doxycycline 200 mg for 4 weeks with topical antibiotics and steroids were administered, the opacity and edema of the cornea regressed after 2 weeks of treatment, and visual acuity in the patient's right eye improved to 20/20. CONCLUSIONS: We report an unusual case of acute interstitial keratitis as the first clinical manifestation of latent syphilis in an immunocompetent patient.