166 results match your criteria: "Fibrous Papule of the Face"

Article Synopsis
  • * Common ocular signs of TS include achromic patches and small retinal tumors (less than 5 mm), and the condition is diagnosed through a combination of clinical features and genetic tests.
  • * A case of a 15-year-old girl with seizures and blurred vision was diagnosed with TS after imaging revealed brain and lung abnormalities, emphasizing the need for early diagnosis and management to prevent complications.
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Tuberous sclerosis complex: A clinical diagnosis in Ethiopian patients.

Medicine (Baltimore)

February 2024

Department of Radiology, College of Medicine and Health Sciences, University of Gondar, Gondar, Ethiopia.

Article Synopsis
  • - Tuberous sclerosis complex (TSC) is a rare genetic disorder causing nonmalignant growths in various organs, with delayed diagnosis often resulting from a lack of physician familiarity.
  • - Two patients, a 27-year-old man with kidney issues and a 28-year-old woman with seizures, were diagnosed with TSC through established diagnostic criteria and exhibited various skin lesions.
  • - Both patients were advised to receive ongoing care: the male was referred to a chronic illness clinic, while the female was scheduled for dermatological treatment for her facial lesions.
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Article Synopsis
  • A 14-year-old cat exhibited symptoms like nosebleeds, facial swelling, reduced appetite, and sneezing, leading to further examinations.
  • Medical imaging revealed a right nasal mass, confirmed as a nasal angiofibroma through tissue analysis.
  • The cat underwent three sessions of radiation therapy, experienced temporary severe mouth sores that healed in 6 weeks, but later showed tumor progression over 21 months.
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'Juvenile' nasal angiofibroma presenting in adulthood.

BMJ Case Rep

June 2023

Department of Otolaryngology-Head and Neck Surgery, Beaumont Hospital, Dublin, Ireland.

Article Synopsis
  • Juvenile nasopharyngeal angiofibromas (JNAs) are rare, benign tumors that are typically found in adolescent males and can cause nasal obstruction and nosebleeds.
  • A case study describes a man in his 30s who presented with nasal obstruction, and after imaging and surgery, was diagnosed with a JNA, despite the unusual age and gender for this condition.
  • The case emphasizes the difficulty in recognizing JNAs in non-adolescent males due to their atypical symptoms and the need for careful diagnostic evaluation.
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Article Synopsis
  • - Tuberous sclerosis is a rare genetic disorder that affects multiple body systems, presenting challenges for both patients and surgeons, particularly regarding facial angiofibromas.
  • - The report discusses two cases: one severe form with skin and neurological issues, and a milder form, both managed through surgical methods like serial excision and electrosurgery.
  • - Effective treatment requires optimizing patient health beforehand and using a multidisciplinary approach to improve safety and outcomes, with combination therapies showing promise.
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Pediatric Endoscopic Endonasal Skull Base Surgery: A Retrospective Review Over 11 Years.

World Neurosurg

February 2023

Division of Neurosurgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, USA; Department of Neurosurgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.

Article Synopsis
  • The study analyzed the safety and effectiveness of the endoscopic endonasal approach (EEA) for pediatric skull base surgery over an 11-year period, involving 94 patients and 130 surgeries.
  • It found that common presenting symptoms included endocrinopathies, vision abnormalities, and cranial nerve deficits, with EEA being employed in the majority of surgeries to treat conditions like craniopharyngioma and pituitary adenoma.
  • Results indicated that EEA is safe with complication rates comparable to adult patients, showing it as a viable option for managing various diseases of the anterior cranial base in children.
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Article Synopsis
  • A case study describes a 59-year-old woman with a 1.5 cm nodule on her lower lip, which was surgically removed and analyzed, showing specific cellular characteristics indicative of extramammary myofibroblastoma.
  • Diagnosis requires differentiating it from similar tumors, and careful examination is crucial to ensure it is not mistaken for malignant conditions; awareness among clinicians and pathologists is essential due to its rare occurrences in unexpected locations.
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Current approach of juvenile nasopharyngeal angiofibroma: a case series.

Rom J Morphol Embryol

September 2022

Department of Otorhinolaryngology, University of Medicine and Pharmacy of Craiova, Romania; Department of Otorhinolaryngology and Ophthalmology, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania;

Article Synopsis
  • Juvenile nasopharyngeal angiofibroma (JNA) is a rare, benign tumor mostly found in males, characterized by its high vascularity.
  • A 3-year study at the Prof. Dr. Dorin Hociotă Institute in Bucharest analyzed eight surgical cases of JNA, all of which underwent pre-surgery imaging and tumor embolization.
  • The majority of cases (seven) were treated using an endoscopic endonasal approach, while one required a combined endonasal-external method, highlighting the importance of a multidisciplinary team for optimal treatment outcomes.
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Tuberous Sclerosis.

Skinmed

December 2021

The Sector of Dermatology and Postgraduation Course in Dermatology, University Hospital and School of Medicine, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil;

Article Synopsis
  • - Tuberous sclerosis is a rare genetic disorder that follows an autosomal dominant pattern, leading to benign tumors called hamartomas in various organs, including the skin, and is mainly monitored by dermatologists.
  • - Key mutations in tumor suppressor genes lead to the development of symptoms, which include multiple skin manifestations like hypomelanotic macules and angiofibromas.
  • - Treatment focuses on managing symptoms, and genetic counseling is crucial, especially since 65% of patients may have no family history due to spontaneous mutations.
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Fibrous papules on the lips.

Pediatr Dermatol

July 2021

Department of Dermatology, Beijing Children's Hospital, Capital Medical University (National Center for Children's Health, China), Beijing, China.

Article Synopsis
  • Multiple angiofibromas are frequently seen in individuals with tuberous sclerosis complex.
  • A rare case is presented where a patient has multiple congenital fibrous papules specifically located on the lips.
  • These lip papules occurred without any associated syndromic conditions, making them an unusual finding.
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Article Synopsis
  • The study examines the midfacial degloving surgical approach for treating large juvenile angiofibromas and compares it to other surgical methods.
  • This retrospective case-series involved 21 male patients, with data collected on their symptoms, surgical details, and postoperative outcomes spanning from 2006 to 2019.
  • Results indicated that the midfacial degloving approach led to a low recurrence rate (14% residual disease) and was effective for patients over 16 years old with extensive angiofibroma involvement.
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Tuberous sclerosis complex is a common neurocutaneous disorder that predominantly affects the brain, skin, eyes, heart and kidneys. The management of tuberous sclerosis complex has been revolutionised with vigabatrin for spasms and everolimus for angiomyolipomas and seizures. We describe a 10-year-old girl with generalised tonic-clonic seizures whose diagnosis of tuberous sclerosis complex was made in view of the presence of a forehead plaque.

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Article Synopsis
  • The study focuses on the inflammatory characteristics observed in fibrous papule (FP), an often-overlooked skin condition, highlighting the presence of inflammation, pagetoid dyskeratosis, and the usefulness of CD163 immunohistochemistry as a diagnostic tool.
  • Researchers analyzed skin biopsy samples of 32 confirmed FP cases for various inflammatory parameters as well as the presence of specific staining patterns in the cells.
  • The findings revealed a high incidence of inflammation in these cases, with approximately 25% showing pagetoid dyskeratosis, and all samples exhibiting prominent CD163 staining, suggesting a potential inflammatory response linked to FP.
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Article Synopsis
  • Juvenile nasopharyngeal angiofibromas (JNAs) are benign but highly vascular tumors that can invade the skull base if not treated promptly, requiring complex surgical techniques.
  • A case study is presented of a 14-year-old boy with a large JNA, where a single-stage combined endoscopic and transoral surgery successfully removed the tumor while minimizing complications.
  • This innovative approach improved surgical access and visualization, enabling complete resection of the tumor without the need for more invasive traditional methods.
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Article Synopsis
  • The study examines papular scars, a newly identified type of acne scarring characterized by dome-shaped papules primarily found on the nose and chin of patients with a history of acne vulgaris.
  • A review of 20 patients revealed that these papules were mostly seen in adolescent Hispanic males, with many also experiencing traditional acne in other facial areas.
  • Histopathological analysis of biopsies from some patients showed features similar to angiofibromas, suggesting that these papules may represent a serious yet under-recognized consequence of acne, especially among adolescents with darker skin tones.
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Cellular angiofibroma of the orbit.

Orbit

August 2021

Department of Oculoplastic, Orbital and Lacrimal Surgery, The Rotterdam Eye Hospital, Rotterdam, The Netherlands.

Article Synopsis
  • * The patient had a mass in his left eye for two years, and imaging tests showed it was a well-defined lesion with specific characteristics on MRI scans.
  • * After surgical removal through the eyelid, the tumor was identified as cellular angiofibroma based on its cellular structure and genetic markers.
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Article Synopsis
  • - Tuberous sclerosis complex (TSC) is a rare genetic disorder that often leads to the development of facial angiofibromas (FAs), which can negatively affect patients' appearance and quality of life (QOL)
  • - A study involving 33 patients assessed the impact of topical sirolimus gel on health-related QOL using the SF-36 survey before and after 3 months of treatment, finding significant improvements in vitality, social function, and mental health
  • - Results indicate that treatment efficacy with sirolimus gel for FAs in TSC patients has a more significant positive impact on QOL compared to any adverse events reported during the study.
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Article Synopsis
  • - Juvenile Nasopharyngeal Angiofibroma is a rare, benign but aggressive tumor that can cause severe symptoms due to its high blood supply, including spontaneous bleeding and nasal obstruction.
  • - A case report details a 13-year-old boy who presented with facial swelling, nasal congestion, recurrent nosebleeds, reduced sense of smell, headaches, and breathing difficulties, with a diagnosis confirmed via CT scan.
  • - The tumor was found to be highly vascularized, and preoperative embolization successfully reduced its blood flow, highlighting the importance of imaging tools for both diagnosis and treatment in managing this condition.
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Article Synopsis
  • Topical rapamycin is known to effectively treat facial angiofibromas (FAs) related to tuberous sclerosis complex (TSC), and calcitriol may also provide benefits by reducing skin fibrosis.
  • A study involving 52 TSC patients tested the effectiveness of a combination of topical rapamycin and calcitriol against single-agent therapies over three 12-week periods, assessing the reduction in severity of FAs.
  • Results showed that while all treatments improved symptoms, the combination therapy outperformed rapamycin alone in terms of faster improvement, greater reduction of skin elevation, and longer-lasting effects, and was well tolerated by patients.
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Article Synopsis
  • Scleromyxedema (SMX) is an inflammatory skin condition that is linked to a type of blood disorder known as monoclonal gammopathy, but its cause is not well understood.
  • The typical features of SMX include a buildup of mucin, increased collagen, and specific fibroblast cells in the skin tissue.
  • This report describes a rare variant of SMX seen in a 41-year-old woman, where facial lesions resembled angiofibromas, highlighting the need to recognize this variant when multiple similar facial lesions with mucin present.
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Article Synopsis
  • - Juvenile nasal angiofibroma (JNA) is a benign but aggressive tumor primarily affecting adolescent boys aged 9-19, accounting for about 0.05% of head and neck tumors.
  • - A study conducted at the N.N. Burdenko National Medical Research Center from 2002 to 2018 involved 143 surgical treatments for JNA in males aged 7-38, with most primary cases found in those under 18.
  • - The paper highlights a rare case of a 38-year-old man diagnosed with pure primary JNA, emphasizing that this tumor can occur in adult men and the importance of accurate diagnosis to avoid confusion in the literature.
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