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Congenital fibrous soft tissue tumors in the eyelids are exceptionally rare, with only seven cases of neonatal myofibromas reported. Myopericytoma, a variant with pericytic differentiation, has not previously been described in neonatal eyelid tumors. This report details a case of a massive congenital myopericytoma in a newborn's lower eyelid. The lesion had a broad area of adhesion to the anterior lamella of the lid. After resection, when the child was 11 days old, the resulting defect was successfully reconstructed with a V-Y type perforator-based flap.

The authors report a patient with nevus of Ota (oculodermal melanocytosis) who presented with pigmented epithelioid melanocytoma in the right orbit. A 36-year-old female patient attended with proptosis OD and diplopia for 3 months. MRI disclosed an expansive lesion adhered to the right inferior rectus muscle, enlargement of the superior orbital fissure, and filling of the right cavernous sinus. Excision of the orbital mass through a transconjunctival inferior fornix access disclosed a heavily pigmented lesion. Anatomopathological study revealed a diagnosis of pigmented epithelioid melanocytoma. A positron emission tomography scan did not show any metastasis. The patient was submitted to radiotherapy, had an improvement of the proptosis and diplopia, and showed no distant metastases for 3 years now. Pigmented epithelioid melanocytoma belongs to the group of heavily pigmented neoplasms, as do atypical blue nevus, which is characterized by prominent melanin production and a low degree of malignancy., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Objective: To evaluate, through a systematic literature review, whether periodontal status in HIV-infected individuals is different from those non-HIV-infected., Materials and Methods: A systematic search for published observational studies within six electronic databases and grey literature was conducted, PROSPERO database number CRD42020160062. Results from studies reporting clinical periodontal parameters: probing pocket depth, bleeding on probing, clinical attachment level, plaque index, and gingival index, in HIV- and non-HIV-infected individuals were reviewed. The quality of the assessment was evaluated according to the Joanna Briggs Institute Appraise Checklist., Results: Twenty-three observational studies met the eligibility criteria and were included for analysis. The qualitative analysis indicated similarities in periodontal parameters within both groups, with no significant mean difference (MD) within both groups regarding clinical periodontal parameters; severe heterogeneity was also detected., Conclusions: No significant differences were found in the periodontal profile of HIV-infected and non-HIV-infected individuals. However, the high heterogeneity among the studies calls for caution in interpreting these findings. Further investigations using standardized methods for periodontal evaluation are needed to clarify the association between HIV infection and periodontal conditions., Competing Interests: The authors declare that there are no conflicts of interest regarding the publication of this article., (Copyright © 2024 Lucas Ribeiro Teixeira et al.)

The study evaluated the regenerative responses of the lacrimal functional unit (LFU) after lacrimal gland (LG) ablation. The LG of Wistar rats was submitted to G1) partial LG ablation, G2) partial ablation and transplantation of an allogeneic LG, or G3) total LG ablation, (n = 7-10/group). The eye wipe test, slit lamp image, tear flow, and histology were evaluated. RT-PCR analyzed inflammatory and proliferation mediators. The findings were compared to naïve controls after 1 and 2 months (M1 and M2). G3 presented increased corneal sensitivity, and the 3 groups showed corneal neovascularization. Histology revealed changes in the LG and corneal inflammation. In the LG, there was an increase in MMP-9 mRNA of G1 and G2 at M1 and M2, in RUNX-1 at M1 and M2 in G1, in RUNX-3 mRNA at M1 in G1, and at M2 in G2. TNF-α mRNA rose in the corneas of G1 and G2 at M2. There was an increase in the IL-1β mRNA in the trigeminal ganglion of G1 at M1. Without changes in tear flow or evidence of LG regeneration, LG ablation and grafting are unreliable models for dry eye or LG repair in rats. The surgical manipulation extended inflammation to the LFU.

Objective: Lysosome-associated membrane protein 3 (LAMP3) misexpression in salivary gland epithelial cells plays a causal role in the development of salivary gland dysfunction and autoimmunity associated with Sjögren's disease (SjD). This study aimed to clarify how epithelial LAMP3 misexpression is induced in SjD., Methods: To explore upstream signaling pathways associated with LAMP3 expression, we conducted multiple RNA sequencing analyses of minor salivary glands from patients with SjD, submandibular glands from a mouse model of SjD, and salivary gland epithelial cell lines. A hypothesis generated by the RNA sequencing analyses was further tested by in vitro and in vivo assays with gene manipulation., Results: Transcriptome analysis suggested LAMP3 expression was associated with enhanced type I interferon (IFN) and IFNγ signaling pathways in patients with SjD. In vitro studies showed that type I IFN but not IFNγ stimulation could induce LAMP3 expression in salivary gland epithelial cells. Moreover, we discovered that LAMP3 overexpression could induce ectopic Toll-like receptor 7 (TLR-7) expression and type I IFN production in salivary gland epithelial cells both in vitro and in vivo. TLR-7 knockout mice did not develop any SjD-related symptoms following LAMP3 induction., Conclusion: Epithelial LAMP3 misexpression can be induced through enhanced type I IFN response in salivary glands. In addition, LAMP3 can promote type I IFN production via ectopic TLR-7 expression in salivary gland epithelial cells. This positive feedback loop can contribute to maintaining LAMP3 misexpression and amplifying type I IFN production in salivary glands, which plays an essential role in the pathophysiology of SjD., (© 2024 The Author(s). Arthritis & Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology. This article has been contributed to by U.S. Government employees and their work is in the public domain in the USA.)

Background: Previous studies have shown that at least a of intraoral eosinophilic ulcer is best classified as a CD30 + T-cell lymphoproliferative disorder (LPD), with histopathology reminiscent of lymphomatoid papulosis (LyP) of the skin. Microscopically, a mixed population of inflammatory cells, often including eosinophils and varying numbers of atypical lymphoid cells, frequently expressing CD30, is typical for LyP, whose clinicopathological spectrum includes type A, B, C, D, E, and LyP with DUSP22/IRF4 rearrangement. To date, about 27 intraoral LyP cases have been reported. Of them, 7 cases were diagnosed as LyP type C, which is frequently confused with anaplastic large cell lymphoma (ALCL) on histopathology., Methods: A 60-year-old male was referred for a one-month history of a tongue ulcer., Results: Microscopy showed numerous subepithelial atypical large lymphoid cells, which expressed CD4 (with partial loss of CD3, CD5, and CD7), CD8 (few cells), CD30 (about 50%, in non-diffuse pattern with size variability), TIA-1, and Ki-67 (85%), without staining for CD56, ALK, LMP1, and EBER1/2, concerning for a diagnosis of ALCL. However, after three weeks, the lesion completely healed., Conclusion: We present here a rare case of intraoral CD30+ T-cell LPD that we believe is the oral counterpart of cutaneous LyP type C., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

An 88-year-old male patient presented with a large mass on the left lateral bulbar conjunctiva. The tumor appeared two months after the resection of a conjunctival atypical fibroxanthoma (AFX) performed by a cornea specialist. Magnetic resonance imaging of the orbits showed deep orbital invasion along the lateral rectus muscle. The mass and the entire conjunctival sac were totally excised with lid-sparing orbital exenteration. Histopathological analysis confirmed that the mass was an extension of the AFX. Two weeks after surgery, large B-cell lymphoma was diagnosed in the oropharynx. Chemotherapy was initiated, and after seven months of follow-up, there was no recurrence of the AFX. The authors believe that this is the first report of orbital invasion by AFX.

Vascular anomalies comprise a wide spectrum of clinical manifestations related to disturbances in the blood or lymph vessels. They correspond to mainly tumors (especially hemangiomas), characterized by high mitotic activity and proliferation of the vascular endothelium, and malformations, endowed with normal mitotic activity and no hypercellularity or changes in the rate of cell turnover. However, the classifications of these lesions go beyond this dichotomy and consist various systems adapted for and by different clinical subgroups. Thus, the classifications have not reached a consensus and have historically caused confusion regarding the nomenclatures and definitions. Cavernous venous malformations of the orbit, previously called cavernous hemangiomas, are the most common benign vascular orbital lesions in adults. Herein, we have compiled and discussed the various evidences, including clinical, radiological, morphological, and molecular evidence that indicate the non-neoplastic nature of these lesions.

Competing Interests: The authors have no financial or conflicts of interest to disclose.

We describe two cases of extensive indolent calvarial osteomyelitis after rhino-orbital-mucormycosis in diabetic patients previously diagnosed with COVID-19. Both patients presented with acute rhino-orbital symptoms about one month after being diagnosed with COVID-19. Treatment with intravenous liposomal Amphotericin B and prompt radical surgical debridement was instituted, but calvarial osteomyelitis ensued and persisted chronically despite maintenance of antifungal therapy and partial debridement of necrotic calvarial bone. The patients were discharged to continue antifungal therapy on a day-hospital regime. After more than 8 months of treatment, they remain with radiological signs of osteomyelitis but with no symptoms or intracranial extension of the infection. Calvarial indolent osteomyelitis secondary to mucormycosis is extremely rare, and little is known regarding its treatment. We believe it can be controlled with medical treatment and partial bony debridement although more studies are necessary to better define therapy.

Progression to aggressive secondary acute myeloid leukaemia (sAML) poses a significant challenge in the management of myeloproliferative neoplasms (MPNs). Since the physiopathology of MPN is closely linked to the activation of interferon (IFN) signalling and that AML initiation and aggressiveness is driven by leukaemia stem cells (LSCs), we investigated these pathways in MPN to sAML progression. We found that high IFN signalling correlated with low LSC signalling in MPN and AML samples, while MPN progression and AML transformation were characterized by decreased IFN signalling and increased LSC signature. A high LSC to IFN expression ratio in MPN patients was associated with adverse clinical prognosis and higher colony forming potential. Moreover, treatment with hypomethylating agents (HMAs) activates the IFN signalling pathway in MPN cells by inducing a viral mimicry response. This response is characterized by double-stranded RNA (dsRNA) formation and MDA5/RIG-I activation. The HMA-induced IFN response leads to a reduction in LSC signature, resulting in decreased stemness. These findings reveal the frequent evasion of viral mimicry during MPN-to-sAML progression, establish the LSC-to-IFN expression ratio as a progression biomarker, and suggests that HMAs treatment can lead to haematological response in murine models by re-activating dsRNA-associated IFN signalling., (© 2023 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.)

Purpose: The study aimed to evaluate the clinical and tissue response to a hollow polymethylmethacrylate orbital implant with a multiperforated posterior surface in an animal model after evisceration., Methods: Sixteen New Zealand rabbits had their right eye eviscerated. All animals received a hollow polymethylmethacrylate implant 12 mm in diameter that is multiperforated in its posterior hemisphere. The animals were divided into four groups, and each one had the eye exenterated at 7, 30, 90, and 180 days post-evisceration. Clinical signs were assessed daily for 14 days post-evisceration and then every 7 days until 180 days. Inflammatory pattern, collagen structure, and degree of neovascularization generated with implant placement were analyzed with hematoxylin-eosin, picrosirius red, and immunohistochemistry staining., Results: There were no signs of infection, conjunctival or scleral thinning, or implant exposure or extrusion in any animal during the study. On day 7, the new tissue migrated into the implant and formed a fibrovascular network through the posterior channels. Inflammatory response reduced over time, and no multinuclea-ted giant cells were found at any time., Conclusion: Hollow polymethylmethacrylate orbital implants with a multiperforated posterior surface enable rapid integration with orbital tissues by fibrovascular ingrowth. We believe that this orbital implant model can be used in research on humans.

We report the case of a 64-year-old male patient with a 5 month history of proptosis, motility limitation and vision loss in OD. Visual acuity (VA) was 20/200 in OD and 20/20 in OS. CT showed a large, round, intraconal lesion, with bony density and no apparent connection to adjacent orbital walls. MRI showed a T1-weighted hypointense lesion surrounded by a contrast enhancing capsule. The orbital tumor was excised through a lateral orbitotomy revealing a nodular, round, osseous structure. Histological examination disclosed well-formed lamellar bone trabeculae, with no necrosis or mitosis figures. Immunohistochemical staining was negative for MDM2 and CDK4. After 3 years, there was no evidence of tumor recurrence and VA had improved to 20/30. Intraconal osteomas with no clear attachment to orbital walls are extremely rare. We are aware of a few reported cases in the lid, hand, thigh, tongue, pterygopalatine fossa and brain. To the authors' knowledge, this is the first report in English literature of an orbital intraconal osteoma without any visible relation to the orbital walls.

Abstract: Lymphomatoid papulosis (LyP) belongs to the spectrum of primary cutaneous CD30 + lymphoproliferative disorders, characterized by chronic, recurrent, self-healing papules, small nodules, or ulcers. The clinicopathological features of LyP can mimic overt lymphomas. To date, about 27 intraoral LyP cases have been reported. Of them, only 2 cases were diagnosed as angioinvasive LyP (type E). Herein, we report a 24-year-old Brazilian man who presented a large ulcerated lesion on the hard palate with rapid evolution. Remarkably, there was no involvement of the skin or other mucous membranes. Microscopy revealed a lymphoid infiltrate constituted by medium-sized to large atypical cells, with angiocentric and angiodestructive features. The atypical cells showed immunopositivity for CD3, CD8, CD30, CD56, granzyme B, perforin, and focally for MUM1/IRF4. Ki-67 highlighted almost all atypical lymphoid cells, whereas EBER1/2 was negative. After 2 months of follow-up, the lesion healed completely. Although rare, LyP type E should be included in the differential diagnosis of oral ulcers., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Congenital opticmeningoceles was the term coined to describe large pseudocystic lesions of the intraorbital segment of the optic nerve. This extremely rare congenital anomaly was reported unilaterally only in nonsyndromic patients with fully developed eyes. The authors describe here a 10-month-old girl with a previous diagnosis of Joubert syndrome who presented with the same type of optic nerve malformation in OU. Molecular genetic analysis disclosed a pathogenic variant of the TMEM67 gene which is associated with various types of ciliopathies., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2023 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Purpose: Xanthogranulomatous pyelonephritis (XGP) is a chronic and severe infection of the kidney. We aimed to review the main clinical, imaging, and histological findings and to assess predictors of surgical complications or hospitalization >10 days (no deaths reported)., Materials and Methods: We retrospectively searched all patients with XGP treated at our institution from 2005 to 2019, with 57 patients enrolled. Clinical data were retrieved by a single reader, computed tomographic (CT) examinations by two radiologists, and histopathological specimens by an experienced pathologist., Results: The patients' mean age was 44.3 ± 16.2 years and 41 (71.9%) were female. The most common symptoms were flank/lumbar pain (89.5%), fever (43.9%), and recurrent urinary tract infection (43.9%). The mean time until the presumptive diagnosis was 365.1 days and the median hospitalization period was 11 days. Blood tests showed anemia (78.9%), leukocytosis (43.6%) with left shift (21.6%). Urinalysis showed hematuria (75.6%), bacteriuria (40.9%), and leukocytes (93.2%). Urine cultures showed Escherichia coli in 14.8%, Proteus mirabilis in 7.4%, while 59.3% were negative. Of 40 patients with CT examinations, 38 (95%) presented with hydronephrosis and perinephric inflammatory changes (PIC) and 22 (55%) with Bear Paw sign. PIC was the only independent predictor at multivariate analysis for surgical complications. For prolonged hospitalization, fever and PIC were independent predictors at univariate, but only fever at multivariate analysis., Conclusions: XGP is a worrisome condition, with unclear pathophysiological mechanisms. Fever and PIC at CT examinations were predictors of poor outcomes., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Urology Annals.)

Background: Intra-arterial chemotherapy (IAC) has been used to treat multiple cancers including liver metastasis from uveal and cutaneous melanoma but not as primary tumor treatment. We report the compassionate use of chemoreduction with intra-arterial melphalan before ruthenium brachytherapy to salvage an eye with choroidal melanoma., Case Presentation: A 61-year-old female patient complained of decreased vision and central-temporal scotoma in OS (left eye) for 1 month. Visual acuity was 20/20 in right eye (OD) and 20/125 OS. Anterior segment examination and intraocular pressure were unremarkable in both eyes, as was fundus examination of the OD. Fundus examination of OS revealed a brown, solid tumor partially obscuring the temporal optic disc margin and extending to the equatorial fundus midzone. Serous retinal detachment was present over the lesion and around it. Ultrasonography revealed a solid choroidal tumor with a largest basal diameter (LBD) of 13.0 mm and thickness of 10.4 mm. The tumor presented acoustic hollowness and a superimposing retinal detachment. After metastatic screening was negative, the patient underwent intra-arterial chemotherapy with melphalan. Three weeks later, her visual acuity was 20/200 and there was noticeable tumor regression to 11.9 mm (LBD) by 7.9 mm (thickness) allowing brachytherapy to be performed. Ten weeks after brachytherapy (13 weeks after IAC), visual acuity was HM due to biopsy-related vitreous hemorrhage (VH). Tumor dimensions were 9.9 (LBD) mm and 6.5 mm (thickness) and PPV was performed to remove VH. Six weeks after PPV (20 weeks after IAC), her visual acuity was 20/200 and further reduction of tumor dimensions was observed: largest basal diameter was 8.9 mm and thickness was 4.9 mm., Conclusion: This case illustrates the feasibility of combining induction IAC prior to ruthenium brachytherapy for large choroidal melanoma. More studies are warranted to confirm these early preliminary findings., (© 2022. The Author(s).)

Kimura's disease (KD) is a systemic inflammatory condition characterized by lymphadenopathy and subcutaneous nodules in the head and neck region. The lesions have a distinctive histopathological pattern formed by follicular hyperplasia, eosinophilic infiltrates, fibrosis, and vessel proliferation. The disease may occur at all ages but predominates among young males with autoimmune dysfunctions. Visceral and orbital involvement is uncommon. We report a girl with KD who developed bilateral enlargement of the lacrimal glands and a lesion in the left lateral ventricle of the brain indistinguishable from a central nervous system neoplasia. A biopsy of both the lacrimal gland and the lateral ventricle was consistent with KD., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2022 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Congenital infiltrating lipomatosis of the face is a rare entity that has not been reported in the ophthalmic literature. The authors describe two patients affected by this condition. Diagnosis and management of congenital infiltrating lipomatosis of the face involving the eye adnexa is challenging and the psychological impact on such patients must be considered. [ J Pediatr Ophthalmol Strabismus . 2022;59(3):e46-e49.] .

Background: Myeloproliferative neoplasms (MPN) are disorders characterized by an alteration at the hematopoietic stem cell (HSC) level, where the JAK2 mutation is the most common genetic alteration found in classic MPN (polycythemia vera, essential thrombocythemia, and primary myelofibrosis). We and others previously demonstrated that metformin reduced splenomegaly and platelets counts in peripheral blood in JAK2 V617F pre-clinical MPN models, which highlighted the antineoplastic potential of biguanides for MPN treatment. Phenformin is a biguanide that has been used to treat diabetes, but was withdrawn due to its potential to cause lactic acidosis in patients., Aims: We herein aimed to investigate the effects of phenformin in MPN disease burden and stem cell function in Jak2 V617F -knockin MPN mice., Results: In vitro phenformin treatment reduced cell viability and increased apoptosis in SET2 JAK2 V67F cells. Long-term treatment with 40 mg/kg phenformin in Jak2 V617F knockin mice increased the frequency of LSK, myeloid progenitors (MP), and multipotent progenitors (MPP) in the bone marrow. Phenformin treatment did not affect peripheral blood counts, spleen weight, megakaryocyte count, erythroid precursors frequency, or ex vivo clonogenic capacity. Ex vivo treatment of bone marrow cells from Jak2 V617F knockin mice with phenformin did not affect hematologic parameters or engraftment in recipient mice., Conclusions: Phenformin increased the percentages of LSK, MP, and MPP populations, but did not reduce disease burden in Jak2 V617F -knockin mice. Additional studies are necessary to further understand the effects of phenformin on early hematopoietic progenitors., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: A scoring system focusing on the risk of muscle layer invasion by Bladder cancer (BCa) has been released, Vesical Imaging - Radiological and Data System (VI-RADS), with a growing interest in evaluating its diagnostic accuracy. Our goal was to assess the accuracy and reproducibility of the VI-RADS score for assessment of the vesical muscular layer with (multiparametric-mp) and without (biparametric-bp) a dynamic-contrast enhancement (DCE) sequence., Methods: Retrospective study conducted from July 2018 to July 2020. All patients had suspicions of BCa and underwent Magnetic Resonance Imaging (MRI) before any intervention. MRI was interpreted by two radiologists with different levels of experience, and a VI-RADS score assigned in two different sessions (3 months apart) without and with DCE. After exclusions, 44 patients with 50 lesions were enrolled. The standard of reference was transurethral resection in 18 patients (40.9%) and cystectomy in 26 patients (59.1%)., Results: Twenty-five lesions (50%) were muscle-invasive. There was no significant difference between the two groups for gender and presence of a stalk, but mean age of NMIBCa group was significantly higher (p = 0.01). The sizes of lesions were significantly different between groups for both readers at 2.42+/- 1.58 vs. 5.70+/- 2.67 cm for reader 1 (p < 0.0001) and 2.37+/- 1.50 vs. 5.44 +/- 2.90 cm for reader 2 (p = 0.001). The area under the curve (AUC) for muscle invasion with mpVI-RADS, considering all lesions, was 0.885 +/- 0.04 (95% CI-0.79-0.98) for reader 1 and 0.924 +/- 0.04 (0.84-0.99) for reader 2, and for bpVI-RADS was 0.879+/- 0.05 and 0.916 +/- 0.04 (0.85-0.99), respectively, both differences not statistically significant (p = 0.24 and 0.07, respectively). When considering only small lesions (< 3.0 cm), the accuracy for mpVI-RADS was 0.795 +/- 0.11 (0.57-1.0) for reader1, and 0.80 +/- 0.11(0.57-1.0) for reader 2, a non-significant difference (p = 0.56) and for bpVI-RADS was 0.747 +/- 0.12 (0.50-0.99) for reader 1 and 0.80 +/- 0.11(0.57-1.0) for reader 2, a significant difference (p = 0.04). The intraclass correlation coefficient for the final score was 0.81 (0.60-1.0) for mpVI-RADS and 0.85 (0.63-1.0) for bpVI-RADS., Conclusion: The VI-RADS system was accurate in demonstrating muscle-invasive BCa, for both experienced and less experienced reader, regardless of the use of a DCE sequence. However, when only small lesions were assessed the difference between the two readers was significant only for the biparametric analysis. The reproducibility was similar between multiparametric and biparametric approach., (© 2022. The Author(s).)

Introduction: Adrenocortical carcinoma (ACC) is diagnosed in paediatric patients at 5 months after symptom onset on average, and 38% die during the first 2.5 years of follow-up. This study aimed to compare the accuracy of Weiss, Van Slooten, and Wieneke histopathological ACC classifications for predicting follow-up prognosis in a paediatric population., Methods: Data were retrieved from medical records of 57 patients aged <18 years who underwent surgical treatment for ACC with surgical follow-up over 6 months or death due to ACC. They were classified into either good (without recurrence/death due to ACC) or poor (with recurrence/death due to ACC) prognosis group. Two expert pathologists classified the ACC surgical specimens according to the Weiss, Van Slooten, and Wieneke criteria., Results: The median follow-up duration was 126 (18-225) months in 38 males (66.7%) and 19 females (33.3%) (median age: 3 [1-6.5] years). The good prognosis group was younger than the poor prognosis group (median age: 3 [1.5-6.2] years vs. 5 [2-10] years). Seventeen (29.8%) patients in the poor prognosis group died due to ACC within the first 50 months of surgical follow-up; the earliest death occurred in the fourth follow-up month, and the majority of deaths occurred within 24 months of follow-up. The accuracies of Weiss, Van Slooten, and Wieneke classification systems were 40%, 47%, and 77%, respectively., Discussion/conclusion: The Wieneke classification showed the best accuracy but was not sufficiently precise to establish reliable prognosis for ACC in the paediatric population. The Wieneke classification had approximately 95% sensitivity and negative predictive value., (The Author(s). Published by S. Karger AG, Basel.)

In acne management, oral isotretinoin (IST) is associated with various untoward systemic effects, while its topical formulation has irritation side effects. Delonix (DLX) is a natural galactomannan derived from Delonix regia seed that can fabricate nanoparticles for topical skin delivery. This study aims to develop IST-DLX nanoparticles and assess their prospects for acne treatment. Fluorescent-DLX nanoparticles (made with a lipophilic BODIPY dye) or IST-DLX nanoparticles were prepared and characterized. BODIPY-DLX nanoparticles' skin distribution and IST-DLX nanoparticles' in-vitro targeting were assessed in pig ear skin, inflammatory modulation was assessed in AMJ-2 macrophage cells, while skin penetration and irritation were assessed in Wistar rats. IST-DLX nanoparticles had ≈230 nm, negative zeta potential, and ≈30% encapsulation efficiency. Confocal showed BODIPY in DLX nanoparticles accumulated in hair follicles as compared to BODIPY solution. IST-DLX nanoparticles released ≈37% IST over 48 h and increased IST 3-fold in hair follicles compared to IST solution. IST-DLX nanoparticles suppressed IL-6 expression in cells and reduced photo-irritation in Wistar rats compared to IST solution. In conclusion, IST-DLX nanoparticles may target and deliver adequate IST to skin layers associated with acne, avoid systemic penetration, modulate inflammatory pathogenic acne stage and prevent IST topical photo-irritation., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Background: To provide basic demographic information and clinicopathologic features of ophthalmic Rosai-Dorfman disease (RDD) with a literature review., Methods: A multi-centre retrospective case series reviewing all patients with histopathologically confirmed ophthalmic RDD at three tertiary eye care centres between January 1993 and December 2018., Results: Eleven eyes of eight patients with histopathologically confirmed ophthalmic RDD were included, with equal numbers of males and females. The median age was 40.25 years (range: 26.6-72.4). Two patients had familial RDD. The orbit was the most commonly involved site (90.9% eyes). One patient (one eye) presented with a scleral nodule, anterior uveitis and cystoid macular oedema. Visual acuity ranged from 20/25 to light perception. Six patients had an extra-nodal ophthalmic disease, and the remaining two had an associated submandibular lymphadenopathy (nodal RDD)., Conclusions: Ophthalmic RDD can be the only manifestation of this systemic disease, with the orbit being the most commonly involved site, exhibiting bone destruction, intracranial and/or sinus involvement and variable degree of visual loss. Ophthalmic familial RDD represent a severe form with a malignant course. Steroid monotherapy may be inadequate to control orbital RDD; thus, combined treatment is usually necessary. A comprehensive approach to assessment and management is recommended., (© 2021. The Author(s).)

Cholesteatomas are frequent middle ear benign tumors of unknown etiology. Infectious agents have been considered as possible contributing factors in the pathogenesis of cholesteatomas. Aiming to investigate the presence of respiratory viruses in primary cholesteatoma tissues, 26 formalin-fixed paraffin-embedded primary cholesteatoma tissues obtained from patients seen at the of the Clinical Hospital of the University of São Paulo School of Medicine, in Ribeirão Preto, Brazil were tested by real-time polymerase chain reaction (PCR). Considering the PCR results, 35% of the tissues were positive for human rhinovirus (HRV), 15.3% for human enterovirus (EV), 3.8% for human metapneumovirus (HMPV), and 3.8% for human bocavirus (HBoV). Serial immunohistochemistry for virus antigens and cell surface markers evidenced that the viruses were associated with fibroblasts, dendritic cells, macrophages, B lymphocytes, CD4 + , and CD8 + T lymphocytes. These findings indicate for the first time the presence of active respiratory virus infection in primary cholesteatoma tissues, suggesting that persisting virus infection in the middle could play a role in the pathogenesis and evolution of cholesteatomas., (© 2021 Wiley Periodicals LLC.)

The association of C-antineutrophil cytoplasmic antibody (ANCA) vasculitis and IgG4 positivity is a new condition not well described in clinical terms. The authors examined a 28-year-old man with a previous diagnosis of eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss disease, who presented with bilateral orbital inflammation. Magnetic resonance imaging revealed diffuse orbital infiltration and enlargement of the major divisions of the trigeminal nerve. Biopsy of the orbital contents showed necrotizing granulomatous vasculitis and a high number of IgG4-positive plasma cells (IgG4/IgG = 60%)., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2021 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)