734 results match your criteria: "Wells Syndrome Eosinophilic Cellulitis"
Wells Syndrome: A Rare Skin Manifestation of Non-Hodgkin's Lymphoma.
Cureus
October 2024
Pathology, Lakeshore Hospital and Research Centre, Ernakulam, IND.
Eosinophilic cellulitis, also known as Wells syndrome, presents a wide range of morphological spectrum, from pruritic erythematous papules, nodules, and pustules to urticarial and bullous lesions. This is a rare dermatological condition and is known to develop after treatment of hematological malignancy. Here, we report a case of Wells syndrome that was the initial presentation of lymphoma, preceding all other symptoms by six months.
View Article and Find Full Text PDFMepolizumab-responsive recurrent eosinophilic eruption without peripheral eosinophilia.
JAAD Case Rep
November 2024
Division of Allergy, Pulmonary and Critical Care Medicine, Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee.
Rapid identification of primary atopic disorders (PAD) by a clinical landmark-guided, upfront use of genomic sequencing.
Allergol Select
October 2024
Center for Child and Adolescent Health, Helios Hospital Krefeld, Academic Hospital of RWTH Aachen, Krefeld.
Article Synopsis
- Primary atopic disorders (PAD) are rare genetic conditions caused by specific gene variants that affect skin and immune function, making diagnosis challenging among common allergic disease cases.
- Identifying PAD requires recognizing clinical red flags like family history and unusual infections, as conventional lab tests are inadequate for definitive diagnosis.
- Whole-genome sequencing (WGS) enhances diagnostic efficiency and accuracy, but requires careful interpretation and collaboration among specialists to effectively manage PAD cases.
Article Synopsis
- * A 41-year-old Turkish woman experienced pruritus and tenderness in her chest, having previously been treated for recurrent abscesses and diagnosed with HES involving multiple organs.
- * Biopsy results indicated eosinophilic infiltration in her rash, and her ongoing respiratory symptoms and childhood asthma hinted at EGPA, highlighting the importance of recognizing these conditions during Wells syndrome investigation.
Recurrent eosinophilic cellulitis (Wells syndrome) after vaccination in a child.
J Dermatol
September 2024
Pediatric Division, Department of Medicine, Academic Hospital S Maria della Misericordia, Udine, Italy.
Wells syndrome following vaccination: A pediatric case with positive patch test to gelatin.
Pediatr Dermatol
August 2024
Department of Dermatology and Venereology, Centro Hospitalar Universitário de São João, Porto, Portugal.
Article Synopsis
- - A 12-month-old boy developed a skin rash 15 days after getting several vaccines, including MMR, pneumococcal, and meningococcal.
- - The skin condition was diagnosed as Wells syndrome, a rare allergic reaction.
- - Patch testing revealed a positive response to gelatin, a common stabilizer found in the MMR vaccine but not in the others.
Hay-Wells syndrome: the challenges of a nine-year follow-up.
An Bras Dermatol
August 2024
Department of Infectology, Dermatology, Imaging Diagnosis and Radiotherapy, Faculty of Medicine, Universidade Estadual Paulista, Botucatu, SP, Brazil.
Toxocariasis and Strongyloidiasis as Triggering Factors for Wells' Syndrome.
Clin Cosmet Investig Dermatol
July 2024
Department of Dermatology and Venereology, Faculty of Medicine, Universitas Padjadjaran, Dr. Hasan Sadikin Hospital, Bandung, Indonesia.
Article Synopsis
- Wells' syndrome is a rare skin condition marked by itchy, red lesions and swelling, often linked to high eosinophil levels and sometimes triggered by parasitic infections.
- A case study in Indonesia reports a 27-year-old male who experienced recurrent painful rashes, fever, and diarrhea, ultimately diagnosed with Wells' syndrome after identifying eosinophilia and conducting histopathological tests.
- The patient was treated for strongyloidiasis with albendazole, leading to improvement within two weeks, highlighting the importance of accurate diagnosis through clinical and pathological evaluations.
Glucose-oxygen deprivation constrains HMGCR function and Rac1 prenylation and activates the NLRP3 inflammasome in human monocytes.
Sci Signal
July 2024
Division of Rheumatology, Department of Endocrinology, Nephrology, Rheumatology, Leipzig University, Leipzig, Germany.
Article Synopsis
- Hypoxia and low glucose levels can occur together during inflammation, causing immune cells like monocytes and macrophages to activate the NLRP3 inflammasome, which produces the inflammatory cytokine IL-1β.
- The activation of the NLRP3 inflammasome in these conditions is linked to the inhibition of HMG-CoA reductase (HMGCR), which is essential for synthesizing a compound called GGPP that helps proteins attach to cell membranes.
- As GGPP synthesis decreases in low glucose and oxygen environments, it leads to impaired protein function, increased activation of the inflammasome, and heightened inflammatory responses, which may contribute to autoimmune diseases.
Article Synopsis
- * A female patient with genetic variants in the IKKα kinase domain exhibited severe immune issues, including low antibody levels and recurrent lung infections, linked to the impaired NF-κB pathways.
- * The study highlights that mutations in IKKα disrupt its function, paving the way for better understanding of NF-κB pathway defects and IKKα's influence on gene expression in humans.
Eosinophilic Dermatosis of Hematologic Malignancy: Emerging Evidence for the Role of Insect Bites-A Retrospective Clinico-Pathological Study of 35 Cases.
J Clin Med
May 2024
Department of Clinical-Surgical, Diagnostic and Pediatric Sciences, University of Pavia, 27100 Pavia, Italy.
Article Synopsis
- Eosinophilic dermatosis of hematologic malignancy (EDHM) is a rare skin disorder linked to blood cancers, especially chronic lymphocytic leukemia, but its causes and prognostic significance are still unclear.
- A study of 35 patients found that 80% were diagnosed with EDHM after their hematologic issues began, and significant proportions experienced disease progression or required treatment.
- The skin lesions exhibited variability and a strong resemblance to insect bites, suggesting potential triggers related to adaptive immune dysfunction, and may also indicate a more severe disease course in patients.
Clinical Entity Mimicking Infectious Cellulitis: Eosinophilic Cellulitis (Wells' Syndrome).
Infect Dis Clin Microbiol
December 2023
Department of Pathology, Health Sciences University Antalya Training and Research Hospital, Antalya, Türkiye.
Article Synopsis
- * Unlike infectious cellulitis, EC does not respond to antibiotics and tends to recur, making its diagnosis challenging.
- * The case report emphasizes the need for skin biopsies to accurately diagnose EC in patients with recurrent cellulitis-like symptoms that don't improve with antibiotics, highlighting its role in differential diagnosis.
Eosinophilic cellulitis (Wells' syndrome) in a female pediatric patient.
Arch Argent Pediatr
August 2024
Department of Dermatology; Department of Pathology; Hospital de Pediatría S.A.M.I.C. Prof. Dr. Juan P. Garrahan, City of Buenos Aires, Argentina.
Article Synopsis
- A case study describes a young girl with asymptomatic subcutaneous nodules and high eosinophil levels, diagnosed with Wells' syndrome through histopathological examination.
- After initial treatment with corticosteroids, which resulted in improvement, the recurrence of symptoms led to the use of dapsone as a second-line therapy, showing positive results.
proteases trigger eosinophil-mediated skin inflammation.
Proc Natl Acad Sci U S A
February 2024
Department of Dermatology, Johns Hopkins School of Medicine, Baltimore, MD 21287.
Article Synopsis
- Skin colonization and eosinophil infiltration are linked to several inflammatory skin disorders, but the role of eosinophils in skin inflammation is not fully understood.
- A mouse model study showed that exposure to certain conditions increased eosinophil-recruiting chemokines and led to notable eosinophil infiltration, contributing significantly to skin inflammation alongside T cells.
- The research identified that IL-36R signaling and proteases are critical in this process, as they promote the recruitment of eosinophils producing IL-17, revealing new insights into how skin inflammation develops in various skin diseases.
A spectrum of TP63-related disorders with eight affected individuals in five unrelated families.
Eur J Med Genet
April 2024
Department of Pediatric Genetics, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
Article Synopsis
- TP63-related disorders involve various symptoms like sparse hair, tooth issues, and cleft lip/palate, linked to mutations in the TP63 gene.
- These disorders include overlapping conditions such as AEC syndrome and EEC3 syndrome, which were diagnosed in patients from five unrelated families.
- Genetic analysis of the TP63 gene revealed multiple variants, indicating a strong genetic link to the observed symptoms, and should prompt doctors to consider these disorders when they see signs of ectodermal abnormalities or clefting.
Article Synopsis
- The study investigates the clinical features of patients with cryopyrin-associated periodic syndrome (CAPS) in Japan and assesses the effectiveness and safety of the IL-1 inhibitor canakinumab in treating these patients.
- Out of 101 patients analyzed, a significant majority achieved complete remission with canakinumab, but 23.7% did not respond, experiencing serious complications including deaths and organ damage.
- The research highlights the importance of early intervention and suggests that combining canakinumab with other therapies can be beneficial for those who don't adequately respond to canakinumab alone.
Neutrophilic and eosinophilic dermatoses associated with hematological malignancy.
Front Med (Lausanne)
January 2024
Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Article Synopsis
- Cutaneous manifestations of blood cancers pose unique challenges for doctors and can provide insights into the diseases themselves, particularly with cases like VEXAS.
- The text reviews various skin conditions related to blood disorders, including Sweet's syndrome and pyoderma gangrenosum, highlighting their clinical significance for both hematologists and dermatologists.
- Management strategies for these skin issues are explored, stressing the importance of treating the underlying blood condition to improve skin symptoms.
Revolutionizing Dupilumab Treatment in Refractory Eosinophilic Cellulitis: A Case Report and Comprehensive Literature Review.
Cureus
December 2023
Internal Medicine, University of Arizona College of Medicine - Phoenix, Phoenix, USA.
Article Synopsis
- Eosinophilic cellulitis (EC), also known as Wells' syndrome, is a rare condition that poses significant challenges in diagnosis and treatment, as illustrated by a case study of a 48-year-old woman with persistent skin lesions.
- Despite various treatment attempts, the patient's dermatitis did not improve until she was treated with dupilumab, a monoclonal antibody that targets interleukin-4 and interleukin-13, which led to significant improvement.
- This case highlights the potential of dupilumab as a breakthrough therapy for EC that does not respond to traditional treatments, emphasizing the need for innovative approaches in managing this complex condition.
Adult patient diagnosed with Muckle-Wells syndrome, antiphospholipid syndrome and glomerular haematuria.
BMJ Case Rep
January 2024
Department of Internal Medicine, Meander Medical Centre, Amersfoort, Utrecht, The Netherlands
Article Synopsis
- Muckle-Wells syndrome (MWS) is a genetic condition that causes periodic fever, skin issues, and hearing loss starting in childhood, which can lead to more serious complications like amyloidosis later in life.
- A 60-year-old patient with a history of periodic fevers, skin reactions to cold, and progressive hearing loss was diagnosed with MWS after genetic testing revealed a harmful gene variant.
- Treatment with anakinra improved his symptoms and reduced blood in urine, highlighting the importance of diagnosing such rare syndromes in adults, as it can lead to effective treatment and prevention of serious complications.
Inflammasome activity is controlled by ZBTB16-dependent SUMOylation of ASC.
Nat Commun
December 2023
Department of Laboratory Medicine, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200025, China.
Article Synopsis
- - Inflammasomes play a key role in immune responses and various clinical conditions, and this study uncovers a specific mechanism regulating the function of Caspase-1 and ASC proteins in their assembly.
- - It was discovered that the nuclear protein ZBTB16 enhances SUMO modification of ASC, which is crucial for proper inflammasome function.
- - The research highlighted the importance of ZBTB16 by showing that removing it in a mouse model reduced inflammation related to a hyperactive inflammasome, providing insight into potential therapeutic targets for inflammatory diseases.
Wells syndrome confined to the back of the hands mimicking contact dermatitis.
J Dermatol
June 2024
Department of Dermatology, Institute of Medicine, University of Tsukuba, Ibaraki, Japan.
A case of cryopyrin-associated periodic syndrome due to somatic mosaic mutation complicated with recurrent circinate erythematous psoriasis.
Mod Rheumatol Case Rep
July 2024
Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Tokyo, Japan.
Article Synopsis
- Cryopyrin-associated periotic syndrome (CAPS) is a rare genetic autoinflammatory disease linked to mutations in the NLRP3 gene, leading to severe inflammation mediated by cytokines like interleukin-1 and interleukin-18.
- The condition has different manifestations based on severity, with other related syndromes identified, making accurate diagnosis difficult due to complex symptoms and the possibility of atypical presentations from somatic mosaic mutations.
- Although there’s no established link between CAPS and psoriasis, there are similarities in immune responses, prompting a case report of CAPS with a somatic mosaic mutation presenting symptoms resembling psoriasis.
Bullous Wells Syndrome Induced by Ustekinumab.
Ann Dermatol
May 2023
Department of Dermatology, School of Medicine, Pusan National University, Busan, Korea.
Bilateral buttock eosinophilic cellulitis after intramuscular benzathine penicillin injection.
Int J Dermatol
January 2024
Fundação de Medicina Tropical Dr Heitor Vieira Dourado (FMT-HVD), Manaus, Brazil.
Ocular involvement in adult and paediatric patients with monogenic autoinflammatory diseases: a Spanish multicentre retrospective study.
Clin Exp Rheumatol
October 2023
Autoinflammatory Diseases Clin Unit & Vasculitis Research Unit, Dept of Autoimmune Diseases, Hosp Clínic Barcelona, Univ of Barcelona, Inst d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Center of the European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA), Spanish Center of the Centros, Servicios y Unidades de Referencia (CSUR) and Catalan Center of the Xarxa d'Unitats d'Expertesa Clínica(XUEC) for Autoinflammatory Diseases, Barcelona, Spain.
Article Synopsis
- * Among 46 patients, conjunctivitis (56.5%) and uveitis (50%) were the most common eye conditions, with cataracts and posterior synechiae being the primary structural complications.
- * The findings indicate that this cohort experienced less severe eye involvement compared to previous studies primarily focused on children, with effective treatment leading to good visual outcomes for most patients.