1,086 results match your criteria: "Degos Disease"
Lupus erythematosus and dermatomyositis.
Clin Dermatol
December 2024
Department of Dermatology, Yale School of Medicine, New Haven, CT. Electronic address:
Dr. Irwin Braverman, a luminary in our field of dermatology, united his love of internal medicine with dermatology to pioneer our understanding of the cutaneous manifestations of systemic disease. His meticulous documentation of physical examination findings in his book Skin Signs of Systemic Disease became fundamental to the training of dermatologists worldwide for decades.
View Article and Find Full Text PDFProposing GLMN, encoding glomulin, as a novel gene for Dowling-Degos disease.
Br J Dermatol
October 2024
Department of Dermatology, Venereology and Allergology, University Medical Center Göttingen, Göttingen, Germany.
Relevance of I-FP-CIT SPECT prescriptions for the diagnosis of parkinsonian syndromes.
Sci Rep
October 2024
Neurology Department, Avicenne Hospital, APHP, Hôpitaux Universitaires de Paris-Seine Saint Denis (HUPSSD), Sorbonne Paris Nord, NS-PARK/FCRIN Network, Bobigny, France.
I-FP-CIT SPECT enables the detection of presynaptic dopaminergic denervation. It allows to differentiate degenerative parkinsonian syndromes from secondary parkinsonian syndromes or essential tremor, and patients with suspected dementia with Lewy bodies from those with other dementia subtypes. The aim of our study was to evaluate the appropriateness of I-FP-CIT SPECT prescriptions, identify prescriber profiles and analyze changes in prescriptions over a decade in the Neurology department of Avicenne University hospital.
View Article and Find Full Text PDFDermatological Enigma Unveiled: A Rare Case Report on Dowling-Degos Disease.
Int J Appl Basic Med Res
August 2024
Department of Microbiology, AIIMS, Rajkot, Gujarat, India.
Article Synopsis
- * It can resemble other skin disorders but is distinguishable by its unique histopathological features.
- * Diagnosis may require a biopsy, as seen in a case where a 50-year-old female was confirmed to have DDD despite no family history or abnormal lab tests, highlighting the need for awareness of rare skin conditions in medical evaluations.
Immediate Effect of Continuous Positive Airway Pressure Therapy on Sleep and Respiration in Patients with Multiple System Atrophy and Sleep-Disordered Breathing.
Mov Disord
November 2024
Service des Pathologies du Sommeil, Pitié-Salpêtrière hospital, AP-HP, Paris, France.
Article Synopsis
- Sleep-disordered breathing (SDB) is common in multiple system atrophy (MSA) patients, and the research evaluated the immediate effects and safety of CPAP therapy for treating SDB.
- In a study of 63 MSA patients, CPAP therapy showed significant improvements such as normalization of apnea-hypopnea index, reduced arousal index, and increased REM sleep, with most patients tolerating the treatment well.
- Nonresponders to CPAP therapy tended to have more severe motor symptoms, indicating that the therapy is effective primarily in milder cases of motor impairment in MSA patients.
Cancer and hidradenitis suppurativa.
Clin Dermatol
December 2024
DermSurgery Associates, Sugar Land, Texas, USA.
Article Synopsis
- * Factors like smoking, prolonged lesions, and certain treatments (like TNF-alpha inhibitors) may increase the risk of developing squamous cell carcinoma in these patients.
- * There is a need for careful monitoring of new or rapidly changing lesions in hidradenitis suppurativa patients, as they can mimic or hide underlying cancers, necessitating prompt medical evaluation.
Deep Brain Stimulation restores information processing in parkinsonian cortical networks.
medRxiv
August 2024
Department of Mathematics and Volen National Center for Complex Systems, Brandeis University, MA Waltham, USA.
Article Synopsis
- Parkinson's Disease (PD) affects neural activity primarily in the basal ganglia and cerebral cortex, and Deep Brain Stimulation (DBS) of the subthalamic nucleus is a key treatment when patients have motor complications from levodopa.
- Research shows that STN-DBS helps improve the brain's ability to transmit information, particularly in predicting limb movements, even if visible EEG patterns don't differ significantly with or without therapy.
- The study examines how different synaptic parameters and excitability levels in cortical networks contribute to enhanced information processing under STN-DBS, suggesting ways to optimize the treatment based on cortical activity measurement.
Tumor necrosis factor ALPHA Inhibitor Associated Köhlmeier-Degos Disease as a Novel Iatrogenic Paradigm That Underscores Excessive Type I Interferon in Its Pathogenesis.
Am J Dermatopathol
September 2024
Sanders Dermatology, New City, NY.
Article Synopsis
- Malignant atrophic papulosis, also known as Köhlmeier-Degos disease, was first identified in 1941 and is characterized by skin rashes and serious intestinal issues, including perforations.
- This disease involves unique vascular damage affecting microvasculature and arteries, leading to distinctive skin lesions and changes in blood vessel structure.
- A case study highlights the emergence of Köhlmeier-Degos disease following treatment with a TNF-alpha inhibitor (golimumab), exploring the relationship between TNF-alpha and type I interferon signaling in the disease's progression.
An Algorithmic Approach Towards Diagnosis of Patients with Hereditary Reticulate Pigmentary Disorders- A narrative review.
Clin Exp Dermatol
August 2024
Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh, India.
Article Synopsis
- * A literature review was conducted to develop a user-friendly diagnostic algorithm to help clinicians identify these disorders and determine appropriate genetic testing.
- * The research included a comprehensive search of databases, resulting in 625 relevant articles that discuss the different diseases and contributed to forming a clear approach for provisional diagnosis based on specific clinical factors.
Case Report: Infant-onset Degos disease with nervous system involvement and a literature review.
Front Pediatr
July 2024
Department of Rheumatology, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, Beijing, China.
Article Synopsis
- Degos disease, or malignant atrophic papulosis (MAP), is an autoinflammatory condition that primarily impacts small- to medium-sized arteries.
- It most frequently affects the gastrointestinal and nervous systems.
- The report discusses a specific case where Degos disease began in infancy, leading to significant neurological complications.
Clinical Manifestation of Degos Disease: Painful Penile Ulcers.
Cutis
April 2024
Baylor Scott & White Health Central Texas Foundation, Temple.
Deleterious effect of sustained neuroinflammation in pediatric traumatic brain injury.
Brain Behav Immun
August 2024
Université Paris Cité, Inserm, NeuroDiderot, 75019 Paris, France.
Article Synopsis
- Pediatric traumatic brain injury (TBI) leads to long-term neuroinflammation and disabilities, especially in children, highlighting the need to study its lasting effects on brain development.
- In a mouse model, researchers induced TBI and then assessed neuroinflammation, myelination, and behavioral changes over time, finding robust inflammation and various neurodevelopmental issues.
- The findings revealed persistent behavioral deficits, such as anxiety and social interaction problems, linked to white matter damage, establishing the mouse model as a valuable tool for future research on TBI outcomes.
Degos disease: A rare disease with a fatal outcome.
Med Clin (Barc)
August 2024
Emergency Physician, Hospital Santa Lucia, Cartagena, Spain.
Clinical Overlaps in Reticulate Pigmentary Disorders: A Study of Three Cases.
Cureus
March 2024
Dermatology, Venereology, and Leprosy, Dr. D. Y. Patil Medical College, Hospital & Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune (Deemed to be University), Pune, IND.
Article Synopsis
- - Reticulate pigmentary disorders are genetic conditions inherited in an autosomal dominant manner, linked to mutations in keratin 5 and keratin 14 genes.
- - The text discusses three cases of patients with reticulate hyperpigmentation disorders showing similarities to reticulate acropigmentation of Kitamura, Dowling-Degos disease (DDD), and dyschromatosis symmetrica hereditaria (DSH).
- - These disorders have challenging treatment options, as all three conditions currently have limited effective treatments available.
Malignant atrophic papulosis treated with eculizumab and hirudin: a fatal case report and literature review.
Front Cardiovasc Med
March 2024
Department of Gastroenterology, The First People's Hospital of Yunnan Province, Kunming, China.
Article Synopsis
- Malignant atrophic papulosis (MAP) is a rare vascular disease with no known cause or established treatment, affecting multiple body systems, particularly the gastrointestinal tract and central nervous system; it generally has a poor prognosis.
- A case study of a 20-year-old male with MAP showed severe symptoms like abdominal pain, bowel perforation, and abscesses, and he was treated with various medications and surgeries but ultimately died within five months.
- The study emphasizes the need for early surgical evaluation and aggressive treatment options like eculizumab and treprostinil for patients with MAP to improve survival rates, particularly those with abdominal symptoms.
Amantadine use in the French prospective NS-Park cohort.
J Neural Transm (Vienna)
July 2024
Department of Clinical Pharmacology and Neurosciences, Clinical Investigation Center CIC1436, Toulouse Parkinson Expert Centre, Toulouse NeuroToul Center of Excellence in Neurodegeneration (COEN), University of Toulouse 3, CHU of Toulouse, INSERM, Toulouse, France.
Article Synopsis
- The study aimed to evaluate the use of amantadine in patients with Parkinson's disease (PD) and its effectiveness in treating levodopa-induced dyskinesia (LIDs).
- It found that 12.6% of PD patients in the French NS-Park cohort were using amantadine, primarily younger patients with more severe symptoms and higher doses of levodopa.
- The results indicated that starting amantadine led to significant improvements in LIDs and motor fluctuations among new users compared to those who had never used the drug.
Contribution of MRI for the Early Diagnosis of Parkinsonism in Patients with Diagnostic Uncertainty.
Mov Disord
May 2024
Assistance Publique Hôpitaux de Paris, Service de Neurologie, Hôpital Avicenne, Hôpitaux Universitaires de Paris Seine-Saint-Denis, Sorbonne Paris Nord, NS-PARK/FCRIN Network, Bobigny, France.
Article Synopsis
- * It involved 113 patients with diagnoses like Parkinson's disease and progressive supranuclear palsy, analyzing the accuracy of clinical criteria, MRI readings, and a machine-learning algorithm.
- * Results indicated that MRI visual reading significantly enhanced diagnostic accuracy by 14.3%, and combining it with clinical criteria further improved accuracy to 85.4%, suggesting MRI is beneficial in early diagnoses.
Galli-Galli Disease: A Comprehensive Literature Review.
Dermatopathology (Basel)
February 2024
Department of Clinical-Surgical, Diagnostic and Pediatric Sciences, University of Pavia, 27100 Pavia, Italy.
Article Synopsis
- Galli-Galli disease (GGD) is a rare genetic skin disorder characterized by red spots, bumps, and a distinct pigmented pattern primarily in skin folds, and presents in both typical and atypical forms.
- The review analyzes cases of GGD since its identification in 1982, covering aspects like causes, how it appears, diagnosis, and treatment options.
- It highlights the similarities between GGD and another skin condition, Dowling-Degos disease (DDD), suggesting they might be different expressions of the same genetic issue, while also noting the lack of effective treatment guidelines for GGD.
A Double-Blind, Randomized, Placebo-Controlled Trial of Bumetanide in Parkinson's Disease.
Mov Disord
March 2024
BA Therapeutics and Neurochlore, Campus Scientifique de Luminy, Marseille, France.
Article Synopsis
- The study explored the potential of bumetanide, a medication aimed at enhancing motor symptoms in Parkinson's disease by targeting GABA-ergic cells.
- It involved 44 participants in a 4-month trial comparing bumetanide to a placebo in conjunction with levodopa treatment.
- The results showed no significant improvement in motor symptoms between bumetanide and placebo groups, and bumetanide was poorly tolerated despite no major safety concerns.
Lymphomatoid Papulosis: A Case Report.
Indian Dermatol Online J
November 2023
Department of Pathology, Lady Hardinge Medical College, New Delhi, Delhi, India.
Article Synopsis
- Lymphomatoid papulosis (LYP) is a chronic skin disorder marked by recurrent, self-healing skin lesions that are benign in appearance but can be malignant on a cellular level.
- Although LYP often resolves on its own, about 10-20% of patients may see it progress to malignant lymphoma, making long-term monitoring important.
- A case study details a 42-year-old male with a 3-year history of recurrent skin lesions on his face and body, healing with discoloration and scars, with no signs of systemic disease and a previous history of treated tuberculosis.
Malignant atrophic papulosis (Degos disease) with central nervous system involvement.
Neurol Sci
April 2024
Department of Neurology, West China Hospital, Sichuan University, No. 37 Guoxue Road, Chengdu, 610041, Sichuan, China.
Article Synopsis
- A 49-year-old man has experienced weakness, sensory issues, bladder/bowel dysfunction, and worsening gait problems for the past two years.
- MRI scans of his brain showed multiple lesions indicating possible damage to small blood vessels in the frontal and parietal lobes.
- The diagnosis of Degos disease (malignant atrophic papulosis) was suggested due to distinctive skin lesions along with complications affecting his central nervous system.
Clinical descriptors of disease trajectories in patients with traumatic brain injury in the intensive care unit (CENTER-TBI): a multicentre observational cohort study.
Lancet Neurol
January 2024
Department of Physiology and Pharmacology, Section of Anaesthesiology and Intensive Care, Department of Physiology and Pharmacology, Karolinska Institutet, Stockholm, Sweden; Function Perioperative Medicine and Intensive Care, Karolinska University Hospital Solna, Stockholm, Sweden. Electronic address:
Article Synopsis
- The study focuses on patients with traumatic brain injury (TBI) in intensive care units (ICUs), aiming to identify clinical variables that predict outcomes after injury.
- Researchers used data from a large European study (CENTER-TBI) involving patients over 18 years old, gathering various pre- and post-injury characteristics and biomarkers.
- The analysis involved a new clustering method to find distinct disease trajectories among ICU patients, linking these clusters to recovery outcomes assessed by the Glasgow Outcome Scale.
Two Phase 3 Trials of Gantenerumab in Early Alzheimer's Disease.
N Engl J Med
November 2023
From the Department of Neurology, Washington University School of Medicine, St. Louis (R.J.B.); Roche Products, Welwyn Garden City (J.S., C.L.), and the Department of Brain Sciences, Faculty of Medicine, Imperial College London (R.J.P.), and the Dementia Research Centre, Department of Neurodegenerative Disease, and the U.K. Dementia Research Institute, Queen Square Institute of Neurology, University College London (N.C.F.), London - all in the United Kingdom; the Alzheimer's Therapeutic Research Institute, Keck School of Medicine, University of Southern California, San Diego (M.C.D.), and Genentech, South San Francisco (T.B., R.S.D.) - both in California; F. Hoffmann-La Roche, Basel, Switzerland (P.D., R.A., J.W., T.B., G.K., A. Thanasopoulou, M. Baudler, P.F., R.S.D.); Butler Hospital and Warren Alpert Medical School, Brown University, Providence, RI (S.S.); the Department of Psychiatry and Neurochemistry, Sahlgrenska Academy, University of Gothenburg, and the Clinical Neurochemistry Laboratory, Sahlgrenska University Hospital - both in Mölndal, Sweden (K.B.); the Division of Neurology, Department of Medicine, Sunnybrook Health Sciences Centre, and the L.C. Campbell Cognitive Neurology Research Unit, Dr. Sandra Black Centre for Brain Resilience and Recovery, Hurvitz Brain Sciences Research Program, Sunnybrook Research Institute, University of Toronto - both in Toronto (S.E.B.); the Ace Alzheimer Center Barcelona, Universitat Internacional de Catalunya, Barcelona, and the Networking Research Center on Neurodegenerative Diseases (CIBERNED), Instituto de Salud Carlos III, Madrid - both in Spain (M. Boada); the Department of Psychiatry and Psychotherapy, Klinikum rechts der Isar, School of Medicine, Technical University of Munich, Munich, Germany (T.G.); the Department of Neurology, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan (A. Tamaoka); the Department of Neurology, Georgetown University School of Medicine, Washington, DC (R.S.T.); the Alzheimer's Research and Treatment Center, Wellington, FL (D.W.); the Medical and Cognitive Research Unit, Heidelberg Repatriation Hospital, Austin Health, Melbourne, VIC, Australia (M.W.); and the Chambers-Grundy Center for Transformative Neuroscience, Department of Brain Health, School of Integrated Health Sciences, University of Nevada, Las Vegas, Las Vegas (J.L.C.).
Article Synopsis
- Monoclonal antibodies like gantenerumab target amyloid-beta to potentially slow decline in Alzheimer’s symptoms, being a fully human anti-Aβ antibody.
- Two phase 3 trials (GRADUATE I and II) involved nearly 2,000 participants aged 50-90 with early signs of Alzheimer’s, comparing gantenerumab with a placebo over 116 weeks.
- Although the trials showed some differences in amyloid levels and cognitive scores between tretament groups, the results were not statistically significant, indicating gantenerumab might not significantly alter cognitive decline compared to placebo.
Trial of Botulinum Toxin for Isolated or Essential Head Tremor.
N Engl J Med
November 2023
From the Department of Neurology, Centre Hospitalier Universitaire (CHU) Clermont-Ferrand, Université Clermont-Auvergne, Centre National de la Recherche Scientifique (CNRS), Image Guided Clinical Neurosciences and Connectomics, Institut Pascal (A.M., P.D., F.D., I.R.), and CHU Clermont-Ferrand, Clinical Research Department, Biostatistics Unit (B.P.), Clermont-Ferrand, the Clinical Investigation Center 1436, the Departments of Clinical Pharmacology, Physiology, and Neurosciences, Toulouse NeuroImaging Center, INSERM, University Hospital of Toulouse and University of Toulouse 3 (M.S.-M., O.R.), and Toulouse NeuroImaging Center, Université de Toulouse, INSERM, Université Paul Sabatier (D.A.), Toulouse, Service de Neurologie, CHU Caremeau, Nîmes (G.C., M.D.V.), the Department of Neurology and Movement Disorders, Timone Hospital, Aix-Marseille Université, Marseille (F.F.), Université de Lyon, Université Claude Bernard Lyon 1, Lyon Neuroscience Research Center, INSERM, Unité 1028, CNRS, Unité Mixte de Recherche (UMR) 5292, Neuroplasticity and Neuropathology of Olfactory Perception Team, Service de Neurologie C, Pierre Wertheimer Neurological Hospital, Hospices Civils de Lyon, Lyon (C.L.), the Neurology Department, Avicenne Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires de Paris-Seine Saint Denis, Sorbonne Paris Nord, Bobigny (B.D.), Centre de Recherche Interdisciplinaire en Biologie, Collège de France, CNRS UMR 7241, INSERM Unité 1050, Université PSL (B.D.), the Neurology Department (A.P.) and Unité Parkinson (S.S.), Rothschild Foundation Hospital, and Sorbonne Université, Institut du Cerveau et de la Moelle, INSERM, CNRS, Assistance Publique-Hôpitaux de Paris, and the Department of Neurology, Hôpital Pitié-Salpêtrière (J.-C.C.), Paris, the Departments of Neurology and Neurosurgery, Expert Center for Parkinson's Disease, EA 4559 Laboratoire de Neurosciences Fonctionnelles et Pathologie, University of Picardy Jules Verne, Amiens University Hospital, Amiens (M.T., D.D.), the University of Lille, Lille Neuroscience and Cognition, Team DVCD, INSERM Unité Mixte de Recherche en Santé 1172, CHU Lille, Department of Medical Pharmacology, Expert Center of Parkinson's Disease, Lille Center of Excellence for Neurodegenerative Disorders, Centers of Excellence in Neurodegeneration, Lille (A.K.), Service de Neurologie, Narbonne Hospital Centre, Narbonne (G.B.-F.), CHU de Bordeaux, Institut des Maladies Neurodégénératives, CNRS, University of Bordeaux, Bordeaux (D. Guehl), the Department of Neurology, University Hospital of Poitiers, INSERM, CHU de Poitiers, University of Poitiers, Centre d'Investigation Clinique 1402, Poitiers (O.C.), the Department of Neuromuscular Diseases and Department of Anatomy, CHRU Besançon, University of Franche-Comté, Besançon (L.T.), Service de Neurologie, Centre Hospitalier Intercommunal Aix-Pertuis, Site d'Aix-en-Provence, Aix-en-Provence (D. Gayraud), and the Department of Neurology, CHU Hautepierre, INSERM Unité 964, CNRS UMR 7104, Illkirch, and Fédération de Médecine Translationnelle de Strasbourg, Université de Strasbourg, Strasbourg (C.T.) - all in France.
Article Synopsis
- Local injections of botulinum toxin type A were tested in a randomized trial to see if they could effectively treat essential head tremor, with participants receiving either the toxin or a placebo.
- The results showed that 31% of patients treated with botulinum toxin experienced significant improvement in their tremors compared to only 9% in the placebo group, indicating a strong positive effect of the treatment.
- However, about half of the patients who received botulinum toxin reported side effects, including neck pain and weakness, highlighting the need for caution despite the treatment's effectiveness.
Is the clinical phenotype impact the prognosis in dementia with Lewy bodies?
Alzheimers Res Ther
October 2023
Cognitive Neurology Center, Université Paris Cité, Lariboisière Fernand Widal Hospital, Assistance PubliqueHôpitaux de Paris, Paris, France.
Article Synopsis
- The study investigates how the initial symptoms of dementia with Lewy bodies (DLB) influence patient outcomes and mortality.
- Conducted at four French neurological centers, the research categorizes patients into groups based on whether their first symptoms were cognitive, psychiatric, or motor.
- Out of 310 patients analyzed, those in the motor group exhibited more severe clinical symptoms, but the initial symptoms did not significantly impact overall mortality rates.