294 results match your criteria: "Malignant Atrophic Papulosis"
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.
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.
Degos disease: A rare disease with a fatal outcome.
Med Clin (Barc)
August 2024
Emergency Physician, Hospital Santa Lucia, Cartagena, Spain.
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.
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.
A loss-of-function NCSTN mutation associated with familial Dowling Degos disease and hidradenitis suppurativa.
Exp Dermatol
November 2023
Keizo Asami Institute-iLIKA, Federal University of Pernambuco, Recife, Brazil.
Article Synopsis
- Dowling Degos disease (DDD) is a rare genetic skin condition that causes progressive pigmented lesions, mainly in skin folds, linked to mutations in several genes, including KRT5, POGLUT-1, and POFUT-1, as well as PSENEN and potentially NCSTN.
- A study involving a family with DDD and hidradenitis suppurativa (HS) identified a new mutation in the NCSTN gene, which results in a premature stop codon and leads to reduced NCSTN protein levels in affected individuals.
- The research suggests that NCSTN is a novel gene associated with DDD and emphasizes the importance of examining its role in patients with both DDD and HS
Malignant Atrophic Papulosis Presenting with Intestinal Perforation: A Case Report.
JNMA J Nepal Med Assoc
June 2023
Department of Internal Medicine, Chhatrapati Free Clinic Hospital, Chhatrapati, Kathmandu, Nepal.
Unlabelled: Malignant atrophic papulosis sometimes known as Degos' disease is an idiopathic, uncommon condition with fewer than 200 occurrences documented. It is a chronic thrombo-obliterative vasculopathy characterised by papular skin lesions with a core porcelain-white atrophy and a surrounding telangiectatic border. We report a 15-year-old male patient with a recurrent history of hollow viscus perforation, which was managed on all the occasions with exploratory laparotomy and primary perforation repair.
View Article and Find Full Text PDFA case of malignant atrophic papulosis with septic complications.
Clin Exp Emerg Med
March 2023
West China School of Medicine, Sichuan University, Chengdu, China.
Increased type I interferon expression from T cells in lesional skin of Degos disease.
J Dermatol
May 2023
Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan.
Atrophic papulosis (Köhlmeier-Degos disease) in children and adolescents-A cross-sectional study and literature review.
J Eur Acad Dermatol Venereol
April 2023
Departments of Dermatology, Venereology, Allergology and Immunology, Staedtisches Klinikum Dessau, Brandenburg Medical School Theodor Fontane and Faculty of Health Sciences Brandenburg, Dessau, Germany.
Article Synopsis
- Atrophic papulosis (Köhlmeier-Degos disease) is a rare vascular condition primarily affecting people aged 20-50 but can occur in children, with a higher male-to-female ratio in pediatric cases.
- A study analyzed data from the Degos Disease Registry, finding that 19 out of 96 patients were children, with median onset age at 5 years, highlighting increased systemic involvement in kids compared to adults.
- Recognizing the specific skin lesions along with neurological or gastrointestinal symptoms is crucial for an accurate diagnosis in pediatric patients, as their presentation may differ from adults.
Malignant atrophic papulosis: Two case reports.
World J Clin Cases
December 2022
Department of Gastrointestinal Surgery, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China.
Article Synopsis
- Malignant atrophic papulosis is a rare and severe microvasculopathy characterized by skin lesions and can lead to gastrointestinal perforations, but its exact cause is still unclear.
- The case study presents two patients: a 67-year-old with skin papules and severe abdominal issues, leading to a diagnosis of the disease after surgery, and a 46-year-old who experienced abdominal pain and complications resulting in death from hemorrhage.
- The findings suggest that the disease can manifest in various ways, challenging researchers to consider it as potentially non-specific due to its impact on multiple organs.
Degos disease in a child presenting with acute renal failure.
Pediatr Dermatol
March 2023
Department of Dermatology, KK Women's and Children's Hospital, Singapore, Singapore.
Article Synopsis
- - Degos disease, also known as malignant atrophic papulosis, is a rare, systemic condition that affects blood flow and is not commonly found in young individuals.
- - The key feature of this disease is a specific type of skin lesion: a papule that has a white, sunken center surrounded by a red, spider-web-like area.
- - A case study highlights an adolescent girl who experienced severe kidney failure due to blood clotting issues caused by the disease, along with the distinctive skin lesion observable through a dermatoscope.
Atrophic Papulosis.
Dermatology
March 2023
Department of Clinical Immunology and Internal Medicine, University of Lyon 1, Hôpital Edouard Herriot, Hospices Civils de Lyon, Lyon, France.
Article Synopsis
- - Atrophic papulosis (AP) is a rare skin vasculopathy leading to specific lesions, with unclear causes and a poor prognosis for its malignant form.
- - The disease can either be benign, limited to the skin, or malignant, which can lead to severe systemic complications and high mortality rates, particularly from gastrointestinal and central nervous system issues.
- - Treatment remains largely empirical, but recent findings suggest that combining eculizumab with treprostinil offers the best results for malignant AP with gastrointestinal involvement.
Degos disease with multiple intestinal perforations: A missed-opportunity case report and literature review.
Front Cardiovasc Med
October 2022
Department of Gastrointestinal Surgery, The First Affiliated Hospital of Shandong First Medical University, Jinan, China.
Article Synopsis
- Degos disease, also known as malignant atrophic papulosis (MAP), is a rare vascular disease affecting multiple systems, particularly the gastrointestinal tract, and is characterized by high mortality due to complications like intestinal perforations.
- A case study details a patient who experienced skin eruptions and multiple intestinal perforations; the diagnosis of Degos disease was delayed despite two surgeries and skin biopsies, ultimately leading to the patient’s death.
- The study highlights the challenges in diagnosing and treating Degos disease, emphasizing the need for increased awareness among medical professionals to improve patient prognosis, especially concerning gastrointestinal complications.
Laparoscopic detection of malignant atrophic papulosis (MAP) in a Paediatric patient.
J Eur Acad Dermatol Venereol
December 2022
Division of Dermatology, Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
Interferon receptor dysfunction in a child with malignant atrophic papulosis and CNS involvement.
Lancet Neurol
August 2022
Department of Pediatric Neurology, Charité-Universitätsmedizin Berlin, 13353 Berlin, Germany; Center for Chronically Sick Children, Charité-Universitätsmedizin Berlin, 13353 Berlin, Germany; Institute for Cell Biology and Neurobiology, Charité-Universitätsmedizin Berlin, 13353 Berlin, Germany.
Inflammation and thrombo-occlusive vessel signalling in benign atrophic papulosis (Köhlmeier-Degos disease).
J Eur Acad Dermatol Venereol
November 2022
Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane and Faculty of Health Sciences Brandenburg, Dessau, Germany.
Background: Although the merely cutaneous, benign form of the extremely rare disease atrophic papulosis (Köhlmeier-Degos disease) may occasionally develop into the systemic, malignant form with time, it is unclear whether it exhibits any systemic characteristics.
Objective: To determine whether benign atrophic papulosis exhibits inflammatory and thrombo-occlusive signals and to classify it according to the Chapel-Hill classification of vasculitis.
Methods: In a monocentric, controlled study, levels of cytokines (IL-1β, IL-6, IL-8, IFNγ, MCP-1, VEGF, TNFα, TGF-β1), antiphospholipid antibodies (cardiolipin IgG/A/M, cardiolipin IgG, cardiolipin IgM, β2-glycoprotein IgG/A/M, phosphatidyl choline, phosphatidyl serine, phosphatidyl inositol, phosphatidyl ethanolamine and sphingomyelin A), antibodies against proteinase-3 IgG and myeloperoxidase IgG, antinuclear antibodies and extractable nuclear antigen were assessed in blood samples of six benign atrophic papulosis patients and six age- and sex-matched healthy controls.
Diffused Degos disease treated with rivaroxaban.
Ital J Dermatol Venerol
June 2022
Department of Dermatology, Hunan Key Laboratory of Skin Cancer and Psoriasis, Hunan Engineering Research Center of Skin Health and Disease, Xiangya Hospital, Central South University, Changsha, China -
Atrophic papulosis (Köhlmeier-Degos disease) revisited: a cross-sectional study on 105 patients.
J Eur Acad Dermatol Venereol
November 2022
Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane and Faculty of Health Sciences Brandenburg, Dessau, Germany.
Background: Atrophic papulosis is a very rare vascular disease of unknown pathogenesis, mostly described by case reports.
Objective: To assess demographic data and prognosis in patients with atrophic papulosis.
Methods: A single-centre study was performed on a series of 105 patients with atrophic papulosis, diagnosed 2000-2021.
Exploring the pathophysiologic basis of constrictive pericarditis of Kohlmeier Degos disease: A case series and review of the literature.
Ann Diagn Pathol
August 2022
Department of Medicine, Albany Medical Center, Albany, NY, United States of America.
Article Synopsis
- - Kohlmeier-Degos Disease is a rare vascular condition that mainly affects the skin, leading to severe complications in the microvasculature and arteries, including life-threatening issues like constrictive pericarditis.
- - In a case study involving three male patients aged 26, 46, and 58, all developed serious cardiac issues within a few years of skin lesions, with two requiring surgical intervention.
- - The disease has a high mortality rate; half of affected patients died within a year of their heart complications, indicating the profound impact of this condition on both skin and cardiovascular health.
Gastrointestinal Kohlmeier-Degos disease: a narrative review.
Orphanet J Rare Dis
April 2022
Department of Surgery, Henry Ford Health System, Detroit, MI, USA.
Article Synopsis
- Kohlmeier-Degos (K-D) disease is a rare vascular condition that can present in a mild cutaneous form or as severe systemic disease, particularly affecting the gastrointestinal tract, leading to high mortality rates due to bowel perforations.
- Symptoms of gastrointestinal K-D often precede perforation and include abdominal pain, weight loss, vomiting, and gastrointestinal bleeding, with the small intestine being the most common site of perforation.
- The most effective treatment involves a combination of eculizumab and treprostinil, and timely diagnostic laparoscopy is crucial for successful diagnosis and intervention.