Eyelid involvement of primary cutaneous lymphomas: A clinical perspective.

Eyelids may be affected in systemic, ocular adnexal and primary cutaneous lymphomas (PCLs). The frequency of eyelid involvement in PCLs is still not well known and it is not a predilection site for any type. While primary cutaneous T-cell lymphomas (CTCLs) are more commonly seen than primary cutaneous B-cell lymphomas (CBCLs), especially mycosis fungoides (MF) as by far the most frequent type, B cell lymphomas are reported to be the commonest type in eyelid localization on the contrary. PCLs may be located on the eyelids, as the sole manifestation or in association with the involvement of other parts of the eye and elsewhere of the body. MF may present with a rich spectrum of clinical features on the eyelids mostly seen in folliculotropic subtype and advanced-stage disease. Erythematous scaly patches or plaques representing the most commonly encountered eyelid MF lesions may mimic many other dermatological conditions. Diffuse thickening, oedema, poikilodermic changes, atrophy and wrinkling are other suggestive findings of eyelid MF. Milia-like papules, madarosis and ectropion are also seen in the folliculotropic variant of MF, as ectropion is more typical for Sezary syndrome. Eyelids are also a typical location for tumoural MF which has been suggested as a poor prognostic indicator in MF. Papulonodular lesions, large tumours, ulceration, diffuse infiltration, oedema and subcutaneous atrophy on the eyelids may also be seen in other types of PCLs. Keep in mind, the rich clinical spectrum of PCLs on the eyelids may be crucial in early diagnosis in this special localization.

Calcium-sensing receptor promotes calcium oxalate crystal adhesion and renal injury in Wistar rats by promoting ROS production and subsequent regulation of PS ectropion, OPN, KIM-1, and ERK expression.

OBJECTIVES: To explore the mechanism of calcium-sensing receptors (CaSRs) during the development of nephrolithiasis. MATERIALS AND METHODS: Wistar rats were treated with ethylene glycol to induce calcium oxalate crystallization, and gadolinium chloride (GdCl3, an agonist of CaSR) and NPS 2390 (an antagonist of CaSR) were added. Oxidative stress (OS) and calcium oxalate crystals in the kidney were observed. CaSR expression and the expression of extracellular signal-regulated protein kinase (ERK), OPN, and KIM-1 were determined by western blotting. In addition, renal tubular epithelial cells were isolated from the kidney to observe phosphatidylserine (PS) ectropion using flow cytometric analysis. Various biochemical parameters were assessed in serum and urine at the end of the experiment. RESULTS: Calcium oxalate increased OS, crystal adhesion, PS ectropion, and the expression of CaSR and ERK, OPN, and KIM-1 in vivo. In addition, lower levels of urine citrate as well as increased serum creatinine and urea levels were observed after treatment with calcium oxalate (p < .05). Compared with calcium oxalate treatment alone, the above deleterious changes were further significantly confirmed by GdCl3 but were reversed by NPS-2390. However, urine calcium excretion was decreased after ethylene glycol treatment but was significantly reduced by NPS 2390 and increased by GdCl3 (p < .05). CONCLUSIONS: The results suggest that CaSR might play significant roles in the induction of nephrolithiasis in rats by regulating reactive oxygen species (ROS) and PS ectropion and the composition of urine, OPN, KIM-1, and ERK expression.

Anterior lamellar deficit ectropion management.

AIMS: To report the outcomes of using a modified Bick's procedure (MBP) combined with a monopedicle myocutaneous flap (MMCF) or full-thickness skin grafting (FTSG) to correct lower eyelid cicatricial ectropion. PATIENTS AND METHODS: A retrospective case-note review of patients undergoing cicatricial ectropion repair between 2012 and 2016 was undertaken. Patient demographics, clinical features, the type of surgery, and outcomes were analysed. RESULTS: Twenty-four eyelids of 21 treated patients (17 males; 81%) with lower eyelid cicatricial ectropion were identified. They presented at an average age of 79.8 years (median 78; range 58-92). The commonest symptom was epiphora (15 eyelids; 63%), 12 patients (50%) experienced intermittent irritation, and 2(8%) had mucoid discharge. The aetiology included actinic cicatricial ectropion with midface descent (n = 19, 79%), previous tumour resection (n = 3, 13%), trauma (n = 1) and other previous eyelid surgery (n = 1). At a mean follow-up period of 15.3 months (median 6; range 6-52), 22 eyelids (92%) had anatomical success with good cosmesis and two eyelids (8%) had mild residual punctal ectropion. Twenty-one patients (87%) experienced functional success. Comparing the outcomes of MBP + FTSG versus MBP + MMCF, there was no statically significant difference in terms of anatomical (p = 0.48) and functional (p = 1.0) success rates. No cases of failure or recurrence were noted during the follow-up period. CONCLUSIONS: Anterior lamellar deficit ectropion occurs in the absence of overt scarring. It is crucial to fully address both the horizontal laxity and the anterior lamellar deficit associated with such ectropion to minimise the risks of early failure and recurrence. MBP combined with FTSG or MMCF is a safe and effective treatment for such 'cicatricial ectropion' and has a low early recurrence rate.

Sight-threatening Complication of Cicatricial Ectropion in a Patient with Lamellar Ichthyosis - Case Report.

We report a case of lamellar ichthyosis and sight-threatening complications of cicatricial ectropion in an adult male patient which was surgically managed with tectonic penetrating keratoplasty. We present a case of autosomal-recessive lamellar ichthyosis in a 47-year-old man who was referred to our outpatient eye clinic for treatment of primary keratouveitis of the right eye with keratolysis and exudation in the anterior chamber. A diagnosis of cicatricial ectropion with serious lagophthalmos was established on examination. The patient underwent tectonic penetrating keratoplasty, cataract extraction, and intra-ocular lens placement with no perioperative complications. The patient was subsequently treated with oral fluconazole 200 mg once daily for 12 days due to a positive fungal culture for Candida albicans and systemic oral acyclovir 250 mg 3 times per day for 12 days as prophylaxis for a labial herpetic infection. Post-operative complications included corneal rejection and nonhealing neurotropic epithelial defect of the graft. Long-term treatment with topical cyclosporine (Ikervis®) and dexamethasone led to resolution of the corneal rejection. Lubrication with artificial tears containing hyaluronic acid, perfluorohexyl octane (Evotears®), and vitamin A ointment led to symptomatic relief of dry eye disease. The patient was referred to a dermatologist and was started on systemic retinoid acitretin at a dose of 0.5 mg/kg per day. Ten months after surgery, the patient's visual acuity was 0.1 based on the Snellen chart and the corneal graft was stable. Infection in the cornea can rapidly progress to corneal melting in patients with severe cicatricial ectropion. A good patient outcome depends on the interdisciplinary approach to patient management by the ophthalmologist, dermatologist, and plastic surgeon.

A theory of unified pathogenesis for tarsal ectropion and involutional entropion.

Tarsal ectropion and involutional entropion are two frequent age-related lower eyelid malpositions with a mirrored clinical presentation. The recent anatomical confirmation of two layers of lower eyelid retractors makes it possible to conceive of the role of each of these layers in the stability of the two palpebral lamellae and their involvement in the pathogenesis of these two malpositions. This study proposes a theory of common pathogenesis involving an involutional change in only the lower lid retractors, leading to the description of two new clinical-anatomical entities.

CDH1-related blepharocheilodontic syndrome is associated with diffuse gastric cancer risk.

We report the first case of diffuse gastric cancer in an individual with familial blepharocheilodontic syndrome (BCD) due to a germline CDH1 likely pathogenic variant. To date, other BCD affected relatives are nonpenetrant for diffuse gastric cancer posing challenges to counseling regarding gastric and breast cancer surveillance, and preventative total gastrectomy.

Diseases, conditions, and drugs associated with cicatricial ectropion / Doenças, condições e drogas associadas ao ectrópio cicatricial

ABSTRACT Cicatricial ectropion may be a consequence of certain systemic diseases as well as the result of drug use. Our goal here was to research the different causes of this condition as reported in the literature, including more recently suspected etiologies. A detailed PubMed literature search indicated many different etiologies were associated with cicatricial ectropion development, from severe cases of systemic diseases, such as ichthyosis and lupus erythematosus, to reversible scenarios secondary to anti-glaucomatous drug use. More recently reported connections include periorbital necrotizing fasciitis, frontal osteomyelitis, and antineoplastic agents. Indeed, cicatricial ectropion may be highly symptomatic; being able to determine its real etiology is imperative to managing patients properly. In this investigation, we felt that an explicitly multidisciplinary approach was essential, especially for cases associated with systemic conditions.

RESUMO O ectrópio cicatricial pode ser uma consequência de certas doenças sistêmicas, bem como o resultado do uso de drogas. Nosso objetivo aqui foi pesquisar as diferentes causas desta condição, conforme relatado na literatura, incluindo mais recentemente suspeitas de etiologias. Uma pesquisa bibliográfica detalhada do PubMed indicou que muitas etiologias diferentes estavam associadas ao desenvolvimento do ectrópio cicatricial, desde casos graves de doenças sistêmicas, como ictiose e lúpus eritematoso, até cenários reversíveis secundários ao uso de drogas antiglaucomatosas. Conexões relatadas mais recentemente incluem fasceíte necrosante periorbitária, osteomielite frontal e agentes antineoplásicos. De fato, o ectrópio cicatricial pode ser altamente sintomático; ser capaz de determinar sua etiologia real é imperativo para o manejo adequado dos pacientes. Nesta investigação, sentimos que uma abordagem explicitamente multidisciplinar era essencial, especialmente para casos associados a doenças sistêmicas.

Diseases, conditions, and drugs associated with cicatricial ectropion.

Cicatricial ectropion may be a consequence of certain systemic diseases as well as the result of drug use. Our goal here was to research the different causes of this condition as reported in the literature, including more recently suspected etiologies. A detailed PubMed literature search indicated many different etiologies were associated with cicatricial ectropion development, from severe cases of systemic diseases, such as ichthyosis and lupus erythematosus, to reversible scenarios secondary to anti-glaucomatous drug use. More recently reported connections include periorbital necrotizing fasciitis, frontal osteomyelitis, and antineoplastic agents. Indeed, cicatricial ectropion may be highly symptomatic; being able to determine its real etiology is imperative to managing patients properly. In this investigation, we felt that an explicitly multidisciplinary approach was essential, especially for cases associated with systemic conditions.

Lower Eyelid Distraction Test: New Insights on the Reference Value.

PURPOSE: The evaluation of palpebral laxity can be performed by the distraction test (DT). Although widely used in ophthalmologic practice, there is no consensus about the results considered normal by the test. The objectives of this study are to obtain the value of DT in a group of individuals with healthy eyelids and to compare with the measurements in subjects with senile ectropion. METHODS: Lower eyelid DT was performed in 200 individuals without any eyelid pathology and in 30 individuals with lower lid ectropion. The results were analyzed by age and sex in the control group and compared with the results of the ectropion group. RESULTS: The mean value of DT in the control group was 6.96 mm, lower than in the ectropion group (9.48 mm) (p < 0.001). In the control group, the mean female DT was 6.70 mm, while the male was 7.22 mm. There were differences in the DT values in the subgroups of 20-39 and 40-59-year old according to gender, with men presenting higher measurements than women. In the other age subgroups, both genders presented similar DT values. Even when considering only individuals in the control group with the same range of age as in the ectropion group, the DT value was higher in individuals with ectropion (7.23 and 9.48 mm, respectively, p < 0.001). CONCLUSIONS: The mean DT value is 6.96 mm; however, the measurement varies according to age and sex in people without palpebral pathology. Individuals with senile eyelid ectropion present higher measurements than healthy ones.In this study, the authors performed the eyelid distraction test in 200 healthy individuals, bringing to the literature the new information that there is a variability of the normal test's value regarding age and gender.

Variants in members of the cadherin-catenin complex, CDH1 and CTNND1, cause blepharocheilodontic syndrome.

Blepharocheilodontic syndrome (BCDS) consists of lagophthalmia, ectropion of the lower eyelids, distichiasis, euryblepharon, cleft lip/palate and dental anomalies and has autosomal dominant inheritance with variable expression. We identified heterozygous variants in two genes of the cadherin-catenin complex, CDH1, encoding E-cadherin, and CTNND1, encoding p120 catenin delta1 in 15 of 17 BCDS index patients, as was recently described in a different publication. CDH1 plays an essential role in epithelial cell adherence; CTNND1 binds to CDH1 and controls the stability of the complex. Functional experiments in zebrafish and human cells showed that the CDH1 variants impair the cell adhesion function of the cadherin-catenin complex in a dominant-negative manner. Variants in CDH1 have been linked to familial hereditary diffuse gastric cancer and invasive lobular breast cancer; however, no cases of gastric or breast cancer have been reported in our BCDS cases. Functional experiments reported here indicated the BCDS variants comprise a distinct class of CDH1 variants. Altogether, we identified the genetic cause of BCDS enabling DNA diagnostics and counseling, in addition we describe a novel class of dominant negative CDH1 variants.

Vertical-to-Horizontal Rotational Myocutaneous Flap for Repairing Cicatricial Lower Lid Ectropion: A Novel Surgical Technique.

OBJECTIVE: To evaluate the efficacy and complications of a novel surgical technique for cicatricial lower lid ectropion that uses a vertical-to-horizontal (V-to-H) rotational myocutaneous flap procedure (Tsai procedure). METHODS: We performed the V-to-H rotational myocutaneous flap procedure on 20 eyelids in 20 patients with mild to moderate cicatricial lower lid ectropion. A vertical myocutaneous flap was created from the anterior lamella of the vertical pedicle in the lateral third of the lower eyelid. Following a horizontal relaxing incision from the base of the flap, a vertical myocutaneous flap was created and rotated to horizontal. Two patients with combined cicatricial ectropion and paralytic lagophthalmos simultaneously underwent additional lateral tarsorrhaphy. RESULTS: After a minimum follow-up period of 6 months, all patients showed good anatomical and functional improvement with decreased dependence on topical lubricants and a satisfactory cosmetic appearance. Two patients with combined cicatricial and paralytic ectropion had mild residual asymptomatic lagophthalmos. No patients required further revision surgery and there were no complications or recurrence. CONCLUSION: The V-to-H rotational myocutaneous flap technique was an effective and simple one-stage procedure for correcting cicatricial lower lid ectropion. It lengthened the anterior lamella and tightened horizontal eyelid laxity without the need for a free skin graft.

Blepharocheilodontic (BCD) syndrome: New insights on craniofacial and dental features.

Blepharocheilodontic (BCD) syndrome is a rare condition characterized by bilateral cleft lip and palate (BCLP), eyelid abnormalities, and oligodontia. Despite orofacial clefting and oligodontia being central features of the condition, detailed reports of dental and craniofacial characteristics are scarce. The aim of this study was to analyze the dental and craniofacial features in a group of patients with BCD syndrome (three of which were related). Cephalometric radiographic analyses were performed on BCD syndrome patients (all radiographs taken at age 8 years) and compared to 40 randomly selected age-matched controls (20 non-syndromic BCLP, 20 non-cleft). Also, we assessed clinical records, photographs, dental study casts, and dental radiographs to determine the extent and pattern of tooth agenesis, dental morphology and malocclusion. BCD syndrome patients showed a very severe skeletal III malocclusion (maxillary-mandibular sagittal discrepancy) and reduced anterior lower face measurement compared to non-syndromic BCLP and non-cleft controls (P = 0.001, P = 0.027). All patients exhibited oligodontia (mean number of missing permanent teeth 13.7, range 7-17). All patients exhibited missing upper central and lateral incisor, upper canine and premolar teeth. Variations in dental morphology included taurodontism, conical-shaped teeth, and notching of the incisal edges. All patients had a short and narrow maxilla which translated into anterior and posterior cross bites. We conclude that, in our BCD syndrome group, the craniofacial skeletal defects are more severe than patients with BCLP. The pattern of tooth agenesis is unusual as it included teeth that are normally highly resistant to agenesis, namely upper central incisor and canine teeth. © 2017 Wiley Periodicals, Inc.

Histopathologic Clues in Diagnosing Oral Mucosal Grafts to the Conjunctiva.

Excised redundant, forniceal "conjunctival" tissue from a 67-year-old man who experienced a chemical injury to his OS 25 years earlier was evaluated histopathologically with the hematoxylin-eosin, periodic acid Schiff (PAS) with and without diastase, mucicarmine, and Alcian blue methods. Additional immunoperoxidase testing for gross cystic disease fluid protein-15 (GCDFP-15) was undertaken. Non-keratinizing squamous epithelium composed of 8 to 10 layers of swollen keratinocytes without goblet cells surmounted a variably dense and well-vascularized collagenized lamina propria deep to which, in submucosal fibroadipose tissue, was embedded an accessory gland. The acini of the gland were composed of both GCDFP-15-positive serous cells and mucicarmine-positive goblet cells, indicating they were seromucinous rather than entirely serous, as is characteristic of normal lacrimal glandular tissue. Different features of the surface epithelium, the lamina propria, and the submucosa can separate the conjunctival and oral mucous membranes. A close analysis of the cytologic composition of associated accessory glands can reinforce the correct diagnosis of an oral mucous membrane graft when the past surgical history is unclear, because only serous cells but not mucocytes comprise the lacrimal glandular units.

Local Recurrence and Ocular Adnexal Complications Following Electronic Surface Brachytherapy for Basal Cell Carcinoma of the Lower Eyelid.

IMPORTANCE: Various treatment options exist for nonmelanoma skin cancer (NMSC), including topical agents, surgery, or definitive or adjuvant radiation therapy. Recently, electronic surface brachytherapy (ESB) has been described as a noninvasive option for NMSC. We report a case of local recurrence of basal cell carcinoma (BCC) and ocular complications following ESB to the lower eyelid. OBSERVATIONS: A man in his 60s presented with a recurrent BCC within the radiation field 10 months after undergoing ESB for a biopsy-proven BCC. In addition to the recurrence, he had contracture of the conjunctiva in the socket of his previously enucleated eye, as well as lower eyelid ectropion, resulting in displacement and loss of retention of his ocular prosthesis. CONCLUSIONS AND RELEVANCE: Electronic surface brachytherapy should be used with caution, particularly in the periocular region because the late effects of hypofractionated radiation may cause ocular and orbital complications. To our knowledge, this is the first reported case of ocular complications with this modality. This case highlights a local recurrence following use of this new treatment modality, suggesting further investigation is warranted to determine the safety and efficacy of ESB.

[Keratopathy versus allergic eye disease: misdiagnosis leading to keratitis].

A case report of involutional ectropion associated with exposure keratopathy - which complicated to exposure keratitis due to incorrect management is presented. Upon consulting the local pharmacy the patient was wrongly given anti-allergic eye drops. Patients with involutional ectropion have high risk of dry eye disease and may rarely be complicated with keratitis. Distinguishing between exposure keratopathy and allergic eye disease is pivotal as management differs and wrong therapy may lead to serious eye disease.