FLOTCH Syndrome: A Case of Leukonychia Totalis and Multiple Pilar Cysts.

FLOTCH (leukonychia totalis-trichilemmal cysts-ciliary dystrophy syndrome) syndrome is a rare genetic cutaneous disorder primarily characterized by multiple recurrent trichilemmal pilar cysts and leukonychia. It may be associated with ciliary dystrophy, koilonychia, and/or less frequently renal calculi and pancreatitis inherited in an autosomal-dominant fashion. We report the case of a 25-year-old Black woman who presented with white-colored fingernails and enlarging cysts in multiple locations including the scalp, rib cage, and forearm and was diagnosed with suspected FLOTCH syndrome. Pilar cysts in unusual locations along with distinct nail changes should prompt clinicians to consider further investigation for conditions such as FLOTCH syndrome.

Chronic eyelid and ocular itch.

Chronic eyelid and ocular itch affect many patients seeking dermatologic or ophthalmologic care and have a high burden on patient quality of life. Clinicians should consider the broad range of possible diagnoses when approaching the patient with itch of the eyes or eyelids lasting more than 6 weeks. Allergic conjunctivitis and allergic contact dermatitis are the most common causes of chronic itch of the eyes and eyelids, respectively. Other diagnoses to consider include atopic dermatitis, xerosis, neurogenic itch, dry eye syndrome, seborrheic dermatitis, blepharitis, rosacea, lichen simplex chronicus, and papulosquamous disorders. If no organic cause can be elucidated, diagnoses of psychogenic pruritus or chronic pruritus of unknown origin may be considered. Herein, we discuss the possible etiologies of chronic eyelid and ocular itch inclusive of clinical presentation, diagnostic considerations, and current therapies.

Efficacy of azithromycin hydrate ophthalmic solution for treatment of internal hordeolum and meibomitis with or without phlyctenular keratitis.

PURPOSE: To evaluate the efficacy of azithromycin hydrate ophthalmic solution for the treatment of internal hordeolum and meibomitis with or without phlyctenular keratitis. STUDY DESIGN: Retrospective study. METHODS: Patients diagnosed with internal hordeolum or meibomitis were prescribed azithromycin hydrate ophthalmic solution twice daily for 2 days and then once daily for 12 days. Depending on the presence of meibomitis-related keratoconjunctivitis (MRKC), we further divided the patients with meibomitis into three subgroups: meibomitis alone (non-MRKC group), meibomitis with non-phlyctenular MRKC (non-phlyctenular group), and meibomitis with phlyctenular MRKC (phlyctenular group). Inflammatory findings (eyelid redness and conjunctival hyperemia) were scored before and after treatment. Some patients also underwent culture testing fluids discharged by the meibomian gland orifices. RESULTS: Three patients (3 eyes) had internal hordeolum and 16 patients (16 eyes) had meibomitis. After treatment, the inflammatory findings disappeared in all eyes with internal hordeolum. Among the patients with meibomitis, three eyes were in the non-MRKC, six in the non-phlyctenular, and seven in the phlyctenular group. The inflammatory findings were significantly improved only in the phlyctenular group. Among seven eyes with positive culture results, Cutibacterium acnes was detected in five, and treatment improved the inflammatory findings in all of these eyes. CONCLUSION: Azithromycin hydrate ophthalmic solution is effective for the treatment of inflammatory meibomian gland diseases, including internal hordeolum and meibomitis. In particular, the agent is highly efficient in patients with phlyctenular MRKC.

Developing and validating a questionnaire to assess the symptoms of blepharitis accompanied by dry eye disease.

PURPOSE: To propose additional items for established dry eye disease (DED) instruments that cover blepharitis-specific signs and symptoms and to determine the association between the clinical findings and subjective complaints. METHODS: Thirty-one patients with blepharitis and DED were prospectively included in the pretest period for selecting suitable questions. In the main phase of the study, the selected questions were then tested on 68 patients with blepharitis and DED and 20 controls without blepharitis or DED. Pearson's coefficient of correlation was calculated between the blepharitis-specific questions, tear break-up time (TBUT), the Schirmer test score, and the ocular surface disease index (OSDI) score; and the similarity between the blepharitis-specific questions, OSDI questions, and objective parameters for DED was assessed via hierarchical clustering. Furthermore, the discriminatory power of the blepharitis-specific questions was investigated with the receiver operating characteristic (ROC) curve. RESULTS: The additional question about heavy eyelids revealed a significant correlation with the OSDI score (r = 0.45, p < 0.001) and Schirmer score (r = - 0.32, p = 0.006). Cluster analysis demonstrated the similarity between the question about heavy eyelids and TBUT. In addition, the OSDI questionnaire had the highest discriminatory power in ROC analysis, and the OSDI score significantly correlated with the specific questions about eyelids sticking together (r = 0.47, p < 0.0001) and watery or teary eyes (r = 0.34, p = 0.003). CONCLUSIONS: The blepharitis-specific additional questions were closely associated with objective parameters for DED. The question about heavy eyelids might be well suited for recording the symptoms of hyposecretory and hyperevaporative dry eye with blepharitis.

Eyelid Exfoliation Treatment Efficacy and Safety in Dry Eye Disease, Blepharitis, and Contact Lens Discomfort Patients: A Systematic Review.

PURPOSE: To determine the efficacy and safety of eyelid exfoliation treatment in dry eye disease (DED), blepharitis, and contact lens (CL) discomfort patients. METHODS: A systematic review that included only full-length randomized controlled studies, reporting the effects of eyelid exfoliation treatment in 2 databases, PubMed and Web of Science, was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses statement. The search period was between October 29, 2022 and December 6, 2022. The Cochrane risk of bias tool was used to analyze the quality of the studies selected. RESULTS: A total of 7 studies were included in this systematic review. Eyelid exfoliation treatment influence on DED, blepharitis, and CL discomfort were analyzed in 6, 4, and 2 studies, respectively. Eyelid exfoliation treatment achieved a better improvement than control group interventions in all reported variables. The mean differences between both groups were as follows: Ocular Surface Disease Index score of -5.0 ± 0.9 points, tear breakup time of 0.43 ± 0.2 seconds, ocular surface staining of -1.4 ± 1.5 points, meibomian glands secretions of 1.2 ± 1.1 points, meibomian glands yielding liquid secretion of 0.6 ± 0.3 points, microorganism load of -3.2 ± 4.7 points, and Contact Lens Dry Eye Questionnaire-8 of -2.15 ± 0.1 points. Minimal discomfort (n = 13) and eyelid irritation (n = 2) were the main complications after an eyelid exfoliation treatment. CONCLUSIONS: Eyelid exfoliation is a safe and effective treatment that should be indicated for DED, blepharitis, and CL discomfort.

BLEPHARITIS AND HELICOBACTER-ASSOCIATED GASTRODUODENAL DISEASES (REVIEW).

The aim of the study was to find the relationship between the inflammatory process on the eyelids (blepharitis) and Helicobascter pylori infection. The data of numerous studies are analyzed, which give grounds to suggest a possible pathogenetic or mediated role of H. pylori infection in the development and course of inflammatory eye diseases. Fundamental in these studies are the release of harmful compounds in the exhaled air of patients with H. pylori, such as ammonia, hydrogen nitrate and hydrogen cyanide, the restoration of impression cytology after H. Pylori eradication, the presence of H. pylori in the lacrimal fluid. In this regard, the authors conclude that there are three main possible pathogenetic mechanisms for the association between H. Pylori infection and blepharitis: chronic inflammation, endothelial dysfunction, and oxidative stress.

Prospective evaluation of intense pulsed light treatment for meibomian gland dysfunction and blepharitis due to ocular rosacea

Background: Ocular rosacea is a common skin condition leading to dry eye that is difficult to manage. Objectives: To estimate the efficacy and safety of a new intense pulsed light device, Thermaeye Plus, for meibomian gland dysfunction and blepharitis due to ocular rosacea. Materials & Methods: This prospective, longitudinal study included 74 eyes of 37 consecutive patients with ocular rosacea, with mean age of 45.6±11.7 years. Four consecutive sessions were undertaken, including14 flashes with 10 J/cm² on the periocular area and facial cheeks on Day 1, 14, 28, and 49. Clinical evaluation was based on: ocular surface disease index (OSDI) and symptom score questionnaires, quality of live and facial severity degree, non-invasive tear meniscus height, non-invasive tear break up time, corneal fluorescein staining and eyelid margin and meibomian gland assessment. Adverse effects on the eye and periocular area, and systemic complications were evaluated. Results: The OSDI questionnaire showed a decrease in symptoms, achieving normal values in 91.9% of patients. The symptom score showed amelioration, with the most significant changes relating to dryness, foreign body sensation, light sensitivity, and pain. Longitudinal analysis showed the most significant improvement between baseline at Day 1 and 49. All eyelid signs improved, most significantly for telangiectasia/vascularity and blepharitis, leading to a 78% clearance of facial rosacea and 81.1% reduction of flushing. In total, 100% of the patients reported an improvement in their quality of life after treatment and 94.6% a very significant improvement (p<0.001). Conclusion: These results demonstrate that Thermaeye Plus is an effective and safe treatment for ocular rosacea.

A Cross-Sectional Study of Ocular Changes in Children and Adolescents with Diabetes Mellitus in Selected Health Facilities in Ghana.

BACKGROUND: The main objective of the study was to determine the prevalence of diabetic retinopathy (DR), other diabetes-related ocular changes (e.g., cataracts, corneal ulceration), and non-diabetic ocular disease in Ghanaian children and adolescents. The second objective was to evaluate the relationship between these conditions and age at diagnosis, current age, diabetes mellitus (DM) duration, and participant's sex. METHODS: A cross-sectional study, undertaken by a multidisciplinary team, included a cohort of children and adolescents (4-19 years) with DM recruited from selected health facilities in Ghana, from March 2016 to September 2019, after written informed consent or assent. The cohort will be followed up for 3 years to determine the natural course of the ocular changes, reported later. Participants were examined for all microvascular and macrovascular complications, non-diabetic ocular disease, anthropometric measurements, laboratory characteristics and quality of life issues. Full ocular examination was also undertaken. Statistical Package for Social Sciences (SPSS Version 25.0) was used for the data analysis. Continuous and categorical variables were presented as mean and standard deviation (SD), median (interquartile range) and as percentages (%), respectively. T-test and Mann-Whitney U test were used in establishing associations. RESULTS: A total of 58 participants were recruited. DR was detected in only 1 out of 58 (1.7%) participants at baseline. Cataracts were the most common ocular finding, detected in 42 (72%) at baseline. Other anterior segment changes observed included blepharitis 46 (79.3%) and tear film instability 38 (65.5%). There was a significant positive association between duration of the DM and the risk of cataract (p = 0.027). Participants' age at diagnosis was significantly associated with the presence of prominent corneal nerves (p = 0.004). CONCLUSIONS: DR was uncommon in this cohort of young persons with DM in Ghana. Cataracts, blepharitis and refractive errors were ocular changes commonly observed. All young persons with diabetes should undergo regular eye examination in all clinics where follow-up care is provided.

Prevalence of Ocular Demodicosis in an Older Population and Its Association With Symptoms and Signs of Dry Eye.

PURPOSE: To evaluate associations between Demodex and its symptoms and ocular surface parameters in individuals with dry eye (DE). METHODS: A cross-sectional study of 119 individuals with DE symptoms or signs, grouped by the presence of ocular demodicosis, was performed. All individuals filled out questionnaires and underwent an ocular surface examination. Demographics, comorbidities, symptoms profiles, and ocular surface parameters were compared between the 2 groups. Multivariable regression analyses were used to determine which factors were associated with DE symptoms. Receiver operating curves analyses were performed to evaluate relationships between Demodex quantity and specific clinical phenotypes. RESULTS: Demodex was highly prevalent in our population (68.9%) with average counts of 4.2 ± 3.9. Individuals with and without Demodex had comparable demographics. Overall, symptoms profiles were similar between individuals with and without Demodex using the Dry Eye Questionnaire 5 and Ocular Surface Disease Index. Individuals with Demodex, however, were more likely to report itching (58.5% vs. 35.1%, P = 0.03), with increasing frequency in those with higher Demodex counts. Individuals with Demodex also had more severe eyelid abnormalities [cylindrical dandruff (CD) and lid vascularity] and a more unstable tear film but similar tear production and corneal staining when compared with individuals without Demodex. Neither Demodex nor ocular surface findings related to DE symptoms in multivariable analyses. Receiver operating curves analysis revealed Demodex counts ≥2 maximized sensitivity and specificity for both itching (64.7% and 59.0%, respectively) and CD (80.4% and 60.4%, respectively). CONCLUSIONS: Demodex is highly prevalent in an older population with DE. Symptom of itching and CD were the findings most specific for Demodex.

Automated Ocular Surface Image Analysis and Health-Related Quality of Life Utility Tool to Measure Blepharokeratoconjunctivitis Activity in Children.

PURPOSE: To explore the tolerability of automated conjunctival hyperemia quantification in children with blepharokeratoconjunctivitis (BKC) and its agreement with clinical activity grading and to explore the Children's Health Utility 9D (CHU9D) as a measure of health-related quality of life in children with BKC. METHODS: We enrolled 63 children, 31 with BKC and 32 without ocular surface inflammation, with a median [interquartile range (IQR)] age of 10.6 (7.2-13.9) years for BKC and 11.4 (9.5-13.8) years for healthy volunteers. Two masked observers graded the ocular surface images. The children indicated discomfort during imaging on a 5-point Likert scale. Using nonparametric tests, we explored the interobserver agreement and the agreement of automated redness index (RI) measurements of limbal and bulbar conjunctival hyperemia with clinician assessment. The children also completed the 9-item CHU9D. RESULTS: The children tolerated imaging well: median (IQR) Likert value of 0 ("comfortable") (0-0) in healthy volunteers and 1 ("a little bit uncomfortable") (0-2) in mild/moderate BKC. In children with BKC, the median (IQR) bulbar RI was 1.3 (0.8-1.6) and the median limbal RI was 0.7 (0.3-1.1). In healthy volunteers, the median bulbar RI was 0.8 (0.55-1.1; P = 0.162) and the median limbal RI was 0.3 (0.2-0.4; P = 0.02). The agreement between RI and clinical grading was high. There was no significant difference between the mean CHU9D utility score between the 2 groups [0.89 (SD 0.08) vs. 0.92 (SD 0.07); P = 0.15]. CONCLUSIONS: Automated conjunctival hyperemia quantification is feasible in children with ocular surface inflammation and may prove useful for long-term monitoring and as an objective outcome measure in clinical trials.

Tectonic keratoplasty using femtosecond laser lenticule in pediatric patients with corneal perforation secondary to blepharokeratoconjunctivitis: a case report and literature review.

Blepharokeratoconjunctivitis secondary to ocular demodicosis in the pediatric population is often neglected and may result in a serious sight-threatening condition. In severe cases, it can lead to corneal perforation necessitating urgent corneal transplantation. However, the shortage and high cost of donor corneas is the foremost limitation of keratoplasty in developing countries. Small-incision lenticule extraction is an advanced flapless femtosecond laser refractive procedure in which an intrastromal corneal lenticule is detached and removed to correct myopia and myopic astigmatism. We herein describe a technique in which lenticules are used for the management of corneal perforation secondary to Demodex-induced blepharokeratoconjunctivitis. The lenticule was sutured over the site of the perforated cornea using 10-0 interrupted nylon sutures. The globe integrity was maintained with a good visual outcome. Thus, tectonic keratoplasty using small-incision lenticule extraction appears to be a safe, cost-effective, and reliable alternative method for the management of corneal perforation secondary to blepharokeratoconjunctivitis.

Demodex Mite Infestation and its Associations with Tear Film and Ocular Surface Parameters in Patients with Ocular Discomfort.

PURPOSE: The presence of Demodex species can be associated with blepharitis. Their pathogenic potential in meibomian gland dysfunction is discussed herein. The purpose of this study was to determine the prevalence of Demodex mites in eyelashes of Austrian patients with ocular discomfort and to evaluate associated changes of the lid margins and meibomian glands. DESIGN: This is a case-control study. METHODS: Two hundred twenty-nine consecutive patients with ocular discomfort from an Austrian dry eye clinic were investigated for the presence of Demodex mites on sampled eyelashes. Associations of a mite infestation with individual dry eye and lid parameters were assessed. Lid margins were evaluated for scales, vascularization, Marx line, expressibility and quality of meibum, and drop-out of meibomian glands. RESULTS: Demodex mites were identified in 40.2% of patients suffering from ocular discomfort (mean mite count 3.3 ± 2.9 per patient). Infestation with mites was associated with the presence of significantly more cylindrical scales (sleeves), a higher Marx line score, and a lower quality of meibum compared with mite-free patients. There were no significant associations with the expressibility and the drop-out of meibomian glands. CONCLUSION: The prevalence of Demodex mites in patients with ocular discomfort is high. The mean mite count per patient in this Austrian dry eye unit population is lower compared with previously published data from Asian regions. The infestation of the eyelids with Demodex species is associated with changes of the anterior and posterior lid margin, suggesting a pathogenic role in blepharitis and meibomian gland dysfunction.

Ocular Demodex folliculorum: prevalence and associated symptoms in an Irish population.

PURPOSE: To investigate the prevalence of ocular Demodex folliculorum (DF) in an Irish population. To validate a modified Ocular Surface Disease Index questionnaire and employ it to evaluate the relationship between dry eye symptoms and the presence of DF. METHODS: One hundred and fifty-six subjects were enrolled in an epidemiological cross-sectional prevalence study. Each subject completed a novel questionnaire on ocular symptoms and was assessed for the presence of DF. Data was analysed to assess prevalence and to search for significant links between each symptom and DF. RESULTS: An overall prevalence of 68% was found. Total mean number of DF found on microscopic examination was 3.83 mites per subject (range 0-25). The presence of symptoms was higher among individuals with DF (P = 0.04). Itch was found to be the symptom most significantly associated with the presence and number of DF (P = 0.025 and P = 0.035, respectively). The questionnaire showed good internal consistency (Cronbach's alpha; α > 0.7) and good reliability (Intra-class Correlation Co-efficient; ICC > 0.7). The sensitivity and specificity of a positive symptom result using the questionnaire were 70.8% and 46.9%, respectively. CONCLUSION: There is a strong association between the prevalence of DF and symptoms, in particular itchy eyes. However, not all patients with DF will be symptomatic. The newly developed questionnaire is a reliable instrument for measuring change in symptoms over a period of time and suitable for observing patient reported outcomes in interventional treatment studies.

Inflamed Obstructive Meibomian Gland Dysfunction Causes Ocular Surface Inflammation.

Meibomian gland dysfunction (MGD) is one of the primary causes of evaporative dry eye. Stagnation of meibum induces an unstable tear film, thus resulting in shortened tear film breakup time and superficial punctate keratopathy (SPK) in the lower cornea and punctate staining of lower bulbar conjunctiva. MGD is sometimes accompanied with inflammation (termed "meibomitis") via the proliferation of bacteria in the meibomian gland and eyelash area. Meibomitis is strongly related to ocular surface inflammation such as corneal cellular infiltrates and neovascularization, SPK, and conjunctivitis. It is difficult to differentiate SPK caused by dry eye from that caused by meibomitis. When clinicians are unaware of the existence of meibomitis, and only aware of SPK on the cornea, they often try to treat SPK as it is caused by dry eye using dry eye-specific eyedrops or even using punctual plugs when conservative therapy is ineffective. However, even when intensive dry eye therapy is applied, it may be unsuccessful until SPK caused by meibomitis is recognized and treated with systemic antimicrobial agents. Hence, the tear secreting glands, including the meibomian glands, and the ocular surface should be clinically considered as one unit (i.e., the meibomian gland and ocular surface [MOS]) when considering the pathophysiology and treatment of ocular surface inflammatory diseases (i.e., corneal epithelial damage). Following this clinical pathway, a treatment focusing on meibomian gland inflammation may be a more reasonable approach for meibomitis-related or associated keratoconjunctivitis to more effectively treat this ocular surface disease.

Investigation of Demodex Parasite Existence in Treatment-Resistant Chronic Blepharitis Cases.

OBJECTIVE: Demodex acari is an obligate ectoparasite, and it is usually located in the human hair follicles, eyelash roots, and sebaceous glands. The aim of this study was to investigate the presence of Demodex infestation in chronic blepharitis cases that are resistant to therapy. METHODS: Patients who were admitted at the Istanbul Medipol University School of Medicine Hospital with a diagnosis of chronic blepharitis were included. All cases received conventional therapy at least three times. Three or four eyelash samples from patients with blepharitis were collected and examined under light microscopy. For the diagnosis, the presence of one or more Demodex parasites at 10× and 40× magnification by a light microscope was considered as positive for infestation. RESULTS: Overall, 153 cases were included in the study. Of the cases, 79 (51.6%) were males and 74 (48.4%) were females. The mean age was 43.1±9.7 years. The presence of D. acari in the follicles of the eyelashes in patients with chronic blepharitis was found in 69 (45.1%) cases. CONCLUSION: D. acari should be considered in patients with chronic blepharitis, especially in treatment-resistant cases. We believe that it would be useful to search for the parasite in patients with blepharitis prior to treatment on a routine basis.

A Rare Case of Blebharitis: Phthiriasis Palpebrarum.

A six-year-old male patient was admitted to our hospital due to itching and scalding crusts that persisted 10-15 days in both eyes. Upon biomicroscopic examination, 5-6 semi-translucent, yellowish brown living lice attached to the upper eyelashes and a large number of eggs were observed. Following application of pilocarpine hydrochloride (Pilomann 2%, Bausch-Lomb) and topical proparacaine hydrochloride (Alcaine 0.5%, Alcon), the paralyzed parasites and eggs were manually removed by pulling with forceps. The lice were identified as adult forms of pubic louse, Pthirus pubis, and its eggs. The patient was treated with pilocarpine hydrochloride, which was applied thrice a day combined with pure vaseline. One week later, no lice or eggs were seen on the eyelashes.

Outcomes of two surgical techniques for major trichiasis treatment.

The purpose of this article is to analyze the outcomes of two surgical techniques to treat major trichiasis. A retrospective chart review of 67 patients (89 eyelids) with major trichiasis was performed who underwent surgical treatment using one of two techniques: intermarginal split lamella with graft (ISLG group) or lid lamella resection (LLR group). There were 30 lids in the ISLG group with mean patient age of 71.8 years and 63.3% were females. There were 59 lids in the LLR group with mean patient age of 72.5 years and 52.5% were female. The minimum postoperative follow up was six months. Statistical analysis included descriptive measures, Goodman association test for contrasts between and within multinomial populations and nonparametric Mann-Whitney test for comparison between groups. P < 0.05 was considered statistically significant. The underlying causes of trichiasis were blepharitis (37.07%), chronic meibomitis (21.3%), multiple causes (20.2%), ectropion (11.2%), actinic keratosis (6.7%), or prior ocular surgery (3.3%). Postoperatively, in the ISLG group, there were 20% lids with complete success, 50% underwent laser or electrolysis, 16.7% required further surgery, and 13.3% were unsuccessful. Postoperatively, in the LLR group, there were 47.5% eyelids with complete success, 46.7% underwent laser or electrolysis, 6.8% required further surgery, and 5.1% were unsuccessful. There was a higher statistical chance of complete success with LLR (P < 0.05). LLR is superior to ISLG surgery for the treatment of major trichiasis. There is a greater chance of success with LLR and it is technically simpler.