-
Journal of the American Academy of... Apr 2024Cystic fibrosis (CF) is caused by a mutation in the Cystic fibrosis transmembrane conductance regulator (CFTR) gene, and features recurrent sinus and pulmonary... (Review)
Review
Cystic fibrosis (CF) is caused by a mutation in the Cystic fibrosis transmembrane conductance regulator (CFTR) gene, and features recurrent sinus and pulmonary infections, steatorrhea, and malnutrition. CF is associated with diverse cutaneous manifestations, including transient reactive papulotranslucent acrokeratoderma of the palms, nutrient deficiency dermatoses, and vasculitis. Rarely these are presenting symptoms of CF, prior to pulmonary or gastrointestinal sequelae. Cutaneous drug eruptions are also highly common in patients with CF (PwCF) given frequent antibiotic exposure. Finally, CFTR modulating therapy, which has revolutionized CF management, is associated with cutaneous side effects ranging from acute urticaria to toxic epidermal necrolysis. Recognition of dermatologic clinical manifestations of CF is important to appropriately care for PwCF. Dermatologists may play a significant role in the diagnosis and management of CF and associated skin complications.
PubMed: 38697219
DOI: 10.1016/j.jaad.2024.04.052 -
JAMA Dermatology Jun 2024
Topics: Humans; Herpesvirus 6, Human; Roseolovirus Infections; Virus Activation; Drug Hypersensitivity Syndrome; Drug Eruptions; Eosinophilia; Latent Infection
PubMed: 38691385
DOI: 10.1001/jamadermatol.2024.0876 -
JAMA Dermatology Jun 2024
Topics: Female; Humans; Male; Middle Aged; Drug Eruptions; Drug Hypersensitivity Syndrome; Eosinophilia; Herpesvirus 6, Human; Latent Infection; Roseolovirus Infections; Virus Activation
PubMed: 38691374
DOI: 10.1001/jamadermatol.2024.0885 -
Clinical Genitourinary Cancer Jun 2024Enfortumab vedotin (EV) is an antibody-drug conjugate approved alone and in combination with pembrolizumab for advanced urothelial cancer (UC)....
INTRODUCTION
Enfortumab vedotin (EV) is an antibody-drug conjugate approved alone and in combination with pembrolizumab for advanced urothelial cancer (UC). EV-related-cutaneous-events (EVCEs) are common and rarely life-threatening. Black patients are frequently under-represented in oncology trials, and dermatologic conditions may vary with race.
METHODS
Therefore, this retrospective analysis investigated differences in EVCE frequency between Black and White patients in an urban cohort (Johns Hopkins [JH]) and a US-based, nationwide electronic health record (EHR)-derived deidentified database (Flatiron Health [FH]) with sub-group analysis of those who had received prior pembrolizumab.
RESULTS
The study included 12 Black patients in the JH Cohort (17.1%) and 24 Black patients in the FH Cohort (7.6%). In both cohorts, the frequency of EVCEs among Black patients was higher compared to White patients (JH: 66.7% vs. 33.3%; FH: 25.0% vs. 15.8%), though not statistically significant. In the larger FH Cohort EVCEs were significantly more common among Black compared to White patients treated with prior pembrolizumab (Odds Ratio [OR]: 4.76 [95%CI: 1.42, 15.95]) and recent pembrolizumab (within 90 days of EV initiation) (OR 9.00 [95%CI: 1.94, 41.66]).
CONCLUSION
This hypothesis-generating retrospective study, comprising the largest population of EV-treated Black patients reported to date, emphasizes the importance of attentiveness to EVCEs among Black patients, particularly with receipt of pembrolizumab.
Topics: Humans; Male; Retrospective Studies; Female; Aged; Antibodies, Monoclonal, Humanized; White People; Middle Aged; Black or African American; Aged, 80 and over; Immunoconjugates; Antineoplastic Combined Chemotherapy Protocols; Drug Eruptions; Urinary Bladder Neoplasms; United States; Antineoplastic Agents, Immunological
PubMed: 38688798
DOI: 10.1016/j.clgc.2024.102090 -
Frontiers in Immunology 2024Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is characterized by a widespread maculopapular rash, lymphadenopathy, fever, and multisystem involvement....
Sulfasalazine-induced drug reaction with eosinophilia and systemic symptoms (DRESS) coinfected with COVID-19 complicated by hemophagocytic lymphohistiocytosis: a case report.
Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is characterized by a widespread maculopapular rash, lymphadenopathy, fever, and multisystem involvement. Conversely, hemophagocytic lymphohistiocytosis (HLH) is an infrequent yet critical condition presenting with fever, hepatosplenomegaly, cytopenias, coagulation abnormalities, and elevated inflammatory markers. The overlapping clinical and laboratory features between DRESS and HLH poses a significant diagnostic challenge. Secondary HLH (sHLH) typically occurs in adults triggered by viral infections, malignancies, rheumatologic diseases, or immune deficiencies. Recently, COVID-19 has also been identified as one of the triggers for sHLH. Herein, we present a case of Sulfasalazine-induced DRESS coinfected with COVID-19 that subsequently progressed into HLH. Our patient exhibited common hepatorenal and splenic involvement along with rare cholecystitis and appendicitis. However, a significant improvement was observed upon the addition of etoposide and azathioprine. We hypothesize that excessive activation of the immune system and cytokine storm due to DRESS combined with COVID-19 infection led to more extensive systemic damage resulting in HLH development. This highlights the potential for severe consequences when DRESS coincides with HLH during a COVID-19 infection.
Topics: Humans; Lymphohistiocytosis, Hemophagocytic; COVID-19; Drug Hypersensitivity Syndrome; Sulfasalazine; SARS-CoV-2; Coinfection; Male; Middle Aged; Female
PubMed: 38686382
DOI: 10.3389/fimmu.2024.1371490 -
The Pan African Medical Journal 2024
Topics: Humans; Anticonvulsants; Carbamazepine; Disease Progression; Stevens-Johnson Syndrome
PubMed: 38681105
DOI: 10.11604/pamj.2024.47.44.42577 -
Annales de Dermatologie Et de... Jun 2024
Topics: Humans; Nivolumab; Dermatitis Herpetiformis; Male; Antineoplastic Agents, Immunological; Drug Eruptions; Female; Pruritus
PubMed: 38678772
DOI: 10.1016/j.annder.2024.103269 -
Annales de Dermatologie Et de... Jun 2024
Topics: Aged; Female; Humans; Antineoplastic Agents, Immunological; Collagen Diseases; Drug Eruptions; Panitumumab
PubMed: 38678770
DOI: 10.1016/j.annder.2024.103271 -
Nature Reviews. Disease Primers Apr 2024Severe cutaneous adverse reactions (SCARs), which include Stevens-Johnson syndrome and toxic epidermal necrolysis, drug reaction with eosinophilia and systemic symptoms... (Review)
Review
Severe cutaneous adverse reactions (SCARs), which include Stevens-Johnson syndrome and toxic epidermal necrolysis, drug reaction with eosinophilia and systemic symptoms (also known as drug-induced hypersensitivity syndrome), acute generalized exanthematous pustulosis, and generalized bullous fixed drug eruption, are life-threatening conditions. The pathogenesis of SCARs involves T cell receptors recognizing drug antigens presented by human leukocyte antigens, triggering the activation of distinct T cell subsets. These cells interact with keratinocytes and various immune cells, orchestrating cutaneous lesions and systemic manifestations. Genetic predisposition, impaired drug metabolism, viral reactivation or infections, and heterologous immunity influence SCAR development and clinical presentation. Specific genetic associations with distinct SCAR phenotypes have been identified, leading to the implementation of genetic screening before prescription in various countries to prevent SCARs. Whilst systemic corticosteroids and conventional immunomodulators have been the primary therapeutic agents, evolving strategies, including biologics and small molecules targeting tumour necrosis factor, different cytokines, or Janus kinase signalling pathways, signify a shift towards a precision management paradigm that considers individual clinical presentations.
Topics: Humans; Stevens-Johnson Syndrome; Drug Hypersensitivity Syndrome; Drug Eruptions; Acute Generalized Exanthematous Pustulosis
PubMed: 38664435
DOI: 10.1038/s41572-024-00514-0