Assuntos
Antineoplásicos , Antivirais , Neoplasias do Ânus , Betapapillomavirus , Carcinoma de Células Escamosas , Infecções por Papillomavirus , Humanos , Antineoplásicos/uso terapêutico , Antivirais/uso terapêutico , Neoplasias do Ânus/tratamento farmacológico , Neoplasias do Ânus/virologia , Carcinoma de Células Escamosas/tratamento farmacológico , Carcinoma de Células Escamosas/virologia , Receptores CXCR4/antagonistas & inibidores , Infecções por Papillomavirus/complicações , Infecções por Papillomavirus/tratamento farmacológico , Infecções por Papillomavirus/virologia , Doenças da Imunodeficiência Primária/tratamento farmacológico , Doenças da Imunodeficiência Primária/genética , Doenças da Imunodeficiência Primária/virologia , Verrugas/tratamento farmacológico , Verrugas/genética , Verrugas/virologia , Mutação com Ganho de FunçãoAssuntos
Linfoma Anaplásico de Células Grandes , Linfoma Anaplásico Cutâneo Primário de Células Grandes , Neoplasias Cutâneas , Quinase do Linfoma Anaplásico/genética , Humanos , Linfoma Anaplásico de Células Grandes/tratamento farmacológico , Linfoma Anaplásico Cutâneo Primário de Células Grandes/tratamento farmacológicoRESUMO
Scleromyxedema is a rare skin and systemic mucinosis that is usually associated with monoclonal gammopathy (MG). In this French multicenter retrospective study of 33 patients, we investigated the clinical and therapeutic features of MG-associated scleromyxedema. Skin molecular signatures were analyzed using a transcriptomic approach. Skin symptoms included papular eruptions (100%), sclerodermoid features (91%), and leonine facies (39%). MG involved an immunoglobulin G isotype in all patients, with a predominant λ light chain (73%). Associated hematologic malignancies were diagnosed in 4 of 33 patients (12%) (smoldering myeloma, n = 2; chronic lymphoid leukemia, n = 1; and refractory cytopenia with multilineage dysplasia, n = 1). Carpal tunnel syndrome (33%), arthralgia (25%), and dermato-neuro syndrome (DNS) (18%) were the most common systemic complications. One patient with mucinous cardiopathy died of acute heart failure. High-dose IV immunoglobulin (HDIVig), alone or in combination with steroids, appeared to be quite effective in nonsevere cases (clinical complete response achieved in 13/31 patients). Plasma cell-directed therapies using lenalidomide and/or bortezomib with dexamethasone and HDIVig led to a significant improvement in severe cases (HDIVig refractory or cases with central nervous system or cardiac involvement). The emergency treatment of DNS with combined plasmapheresis, HDIVig, and high-dose corticosteroids induced the complete remission of neurological symptoms in 4 of 5 patients. Quantitative reverse-transcriptase polymerase chain reaction analysis of 6 scleromyxedema skin samples showed significantly higher profibrotic pathway levels (transforming growth factor ß and collagen-1) than in healthy skin. Prospective studies targeting plasma cell clones and/or fibrotic pathways are warranted for long-term scleromyxedema management.
Assuntos
Paraproteinemias/complicações , Paraproteinemias/terapia , Plasmócitos/patologia , Escleromixedema/complicações , Escleromixedema/terapia , Adulto , Idoso , Antineoplásicos/uso terapêutico , Bortezomib/uso terapêutico , Dexametasona/uso terapêutico , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Lenalidomida/uso terapêutico , Masculino , Pessoa de Meia-Idade , Paraproteinemias/genética , Paraproteinemias/patologia , Plasmócitos/efeitos dos fármacos , Plasmócitos/metabolismo , Plasmaferese , Estudos Retrospectivos , Escleromixedema/genética , Escleromixedema/patologia , Pele/metabolismo , Pele/patologia , TranscriptomaAssuntos
Quimiorradioterapia/efeitos adversos , Eritema Nodoso/etiologia , Síndrome de Sweet/etiologia , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Neoplasias do Ânus/terapia , Eritema Nodoso/diagnóstico , Feminino , Fluoruracila/administração & dosagem , Fluoruracila/efeitos adversos , Humanos , Pessoa de Meia-Idade , Mitomicina/administração & dosagem , Mitomicina/efeitos adversos , Síndrome de Sweet/diagnósticoAssuntos
Predisposição Genética para Doença/genética , Leucemia/genética , Síndromes Mielodisplásicas/genética , Xeroderma Pigmentoso/genética , Cariótipo Anormal , Adolescente , Adulto , Criança , Reparo do DNA/genética , Proteínas de Ligação a DNA/genética , Feminino , Efeito Fundador , Genes p53/genética , Humanos , Masculino , Mutação , Proteína Supressora de Tumor p53/genética , Xeroderma Pigmentoso/complicações , Adulto JovemAssuntos
Adenosina Desaminase/deficiência , Adenosina Desaminase/genética , Peptídeos e Proteínas de Sinalização Intercelular/deficiência , Peptídeos e Proteínas de Sinalização Intercelular/genética , Úlcera da Perna/genética , Dermatopatias Genéticas/diagnóstico , Adulto , Doença Crônica , Consanguinidade , Humanos , Úlcera da Perna/patologia , Masculino , Linhagem , Dermatopatias Genéticas/genéticaAssuntos
Antineoplásicos Imunológicos/efeitos adversos , Carcinoma de Células Escamosas/terapia , Neoplasias Pulmonares/terapia , Nivolumabe/efeitos adversos , Radiodermite/etiologia , Síndrome de Stevens-Johnson/etiologia , Carcinoma de Células Escamosas/secundário , Humanos , Neoplasias Pulmonares/patologia , Masculino , Pessoa de Meia-Idade , Radioterapia/efeitos adversosAssuntos
Cútis Laxa/epidemiologia , Cútis Laxa/patologia , Paraproteinemias/epidemiologia , Paraproteinemias/patologia , Adulto , Distribuição por Idade , Idoso , Biópsia por Agulha , Comorbidade , Feminino , Humanos , Imuno-Histoquímica , Incidência , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos de Amostragem , Índice de Gravidade de Doença , Distribuição por SexoAssuntos
Carcinoma de Células Escamosas/complicações , Líquen Plano/complicações , Neoplasias Cutâneas/complicações , Úlcera Cutânea/complicações , Adulto , Idoso , Carcinoma de Células Escamosas/patologia , Feminino , Doenças do Pé/complicações , Mãos , Humanos , Masculino , Neoplasias Cutâneas/patologiaAssuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Antineoplásicos Imunológicos/efeitos adversos , Colite Ulcerativa/induzido quimicamente , Colite Ulcerativa/tratamento farmacológico , Infliximab/efeitos adversos , Ipilimumab/efeitos adversos , Idoso , Antineoplásicos Imunológicos/uso terapêutico , Colite Ulcerativa/diagnóstico por imagem , Doença de Crohn/induzido quimicamente , Doença de Crohn/diagnóstico por imagem , Humanos , Infliximab/uso terapêutico , Ipilimumab/uso terapêutico , Masculino , Melanoma/complicações , Melanoma/tratamento farmacológicoAssuntos
Fibrossarcoma/etiologia , Joias/toxicidade , Neoplasias Induzidas por Radiação/patologia , Neoplasias de Tecidos Moles/etiologia , Tórax/patologia , Idoso de 80 Anos ou mais , Evolução Fatal , Fibrossarcoma/patologia , Humanos , Masculino , Metástase Neoplásica , Esclerose/etiologia , Neoplasias de Tecidos Moles/patologia , Tório/toxicidade , Urânio/toxicidadeRESUMO
BACKGROUND: Biotherapies or targeted therapies are fairly new treatments indicated for moderate to severe psoriasis. The side effects appear to be mainly infectious or cancerous. The role of biotherapies in the development of a pre-cancerous condition, monoclonal gammopathy of undetermined significance (MGUS), has recently been debated in the literature. OBJECTIVES: To evaluate the incidence of MGUS in psoriasis patients treated with biotherapy. MATERIALS AND METHODS: This study was a French multicenter retrospective study carried out through the French multicenter study group RESOPSO. Data on the results of serum protein electrophoreses performed before and within at least six months after the start of the biotherapy were collected. Demographic data, medical history, and psoriasis treatment history were specified. RESULTS: Four hundred and forty three patients were eligible for inclusion. Of these, three presented with monoclonal gammopathy for which the assessment was in favor of MGUS. The average treatment period was 19.7 months. Six patients presented with MGUS prior to the treatment. These patients' immunoglobulin levels remained stable, with an average remission of 24 months. Only psoriatic rheumatism appeared to be statistically linked to MGUS. CONCLUSION: The incidence and frequency of MGUS in psoriasis patients treated with biotherapy do not appear to increase relative to the general population.
Assuntos
Fatores Biológicos/efeitos adversos , Paraproteinemias/etiologia , Psoríase/terapia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Psoríase/imunologia , Estudos RetrospectivosRESUMO
OBJECTIVES: The broader and prolonged use of anti-tumor necrosis factor (TNF) agents in inflammatory bowel disease (IBD) could expose patients to an increased risk of adverse reactions, including dermatological complications. We assessed the cumulative incidence of anti-TNF-induced cutaneous adverse reactions in IBD patients, their risk factors, their dermatological management, and their outcome in a large cohort of IBD patients. METHODS: In a single-center observational retrospective study, including all consecutive adult IBD patients treated with an anti-TNF agent between 2001 and 2014, all patients with dermatological complications under anti-TNF therapy were identified in a well-defined cohort of IBD patients. We conducted a survival analysis to determine the cumulative incidence of dermatological complications and risk factors for developing any dermatological complications, cutaneous infections, and psoriasiform lesions. Survival curves were estimated by the Kaplan-Meier method, and we used a Cox proportional hazards model to test the association between parameters and time to each event: any dermatological complication, cutaneous infections, and psoriasis lesions. RESULTS: Among 583 IBD patients, 176 dermatological complications occurred, involving 20.5% of patients. Median duration of follow-up was 38.2 months (range: 1-179). Psoriasiform lesions (10.1%; 59/583) and cutaneous infections (11.6%, 68/583) were the most frequently observed, with a cumulative incidence of, respectively, 28.9% and 17.6% at 10 years. They led to anti-TNF discontinuation, respectively, in 18.6% and 2.9% of patients. In case of switching to another anti-TNF agent for psoriasiform lesions, recurrence occurred in 57% of patients. Ulcerative colitis was associated with a lower risk of developing cutaneous infections than Crohn's disease (hazard ratio (HR)=0.25; 95% confidence interval (CI)=0.09-0.68; P=0.007). Higher dosing of anti-TNF agent was associated with a higher risk of developing cutaneous infections (HR=1.99; 95% CI=1.09-3.64; P=0.025). A younger age at time of anti-TNF initiation was associated with a higher risk of dermatological complications (HR=2.25; 95% CI=1.39-3.62; P<0.001). CONCLUSIONS: Dermatological complications involve one of five patients treated with anti-TNF therapy after a 14-year follow-up. Association of cutaneous infections with higher anti-TNF dosing suggests a dose-dependent effect. Discontinuation of anti-TNF therapy due to dermatological complications is required in one out of five patients with psoriasiform lesions, but specific dermatological treatment allows to continue anti-TNF therapy in half of them.
Assuntos
Anti-Inflamatórios não Esteroides/efeitos adversos , Colite Ulcerativa/tratamento farmacológico , Doença de Crohn/tratamento farmacológico , Toxidermias/epidemiologia , Psoríase/epidemiologia , Dermatopatias Infecciosas/epidemiologia , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adalimumab , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Anti-Inflamatórios não Esteroides/administração & dosagem , Anticorpos Monoclonais/administração & dosagem , Anticorpos Monoclonais/efeitos adversos , Anticorpos Monoclonais Humanizados/administração & dosagem , Anticorpos Monoclonais Humanizados/efeitos adversos , Criança , Relação Dose-Resposta a Droga , Toxidermias/etiologia , Toxidermias/patologia , Feminino , Humanos , Incidência , Infliximab , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Prevalência , Modelos de Riscos Proporcionais , Psoríase/induzido quimicamente , Psoríase/tratamento farmacológico , Estudos Retrospectivos , Fatores de Risco , Dermatopatias Infecciosas/induzido quimicamente , Dermatopatias Infecciosas/tratamento farmacológico , Adulto JovemAssuntos
Blefarite/etiologia , Neoplasias da Mama/secundário , Neoplasias da Túnica Conjuntiva/secundário , Conjuntivite/etiologia , Neoplasias Palpebrais/diagnóstico , Idoso , Biópsia , Blefarite/diagnóstico , Neoplasias da Mama/diagnóstico , Neoplasias da Túnica Conjuntiva/diagnóstico , Conjuntivite/diagnóstico , Diagnóstico Diferencial , Neoplasias Palpebrais/complicações , Feminino , HumanosRESUMO
BACKGROUND: UV radiation protection is an important health issue. Sophisticated sunscreen formulations have been developed to improve compliance. However, sunscreen is still inadequately applied, leaving large body areas without effective protection. AIM: This study aims to validate a newly developed sunscreen application technique for adults and children. METHODS: Fifty-eight volunteers were recruited to participate in a monocenter, intraindividual, sequential, comparative study. The covering potential of their currently used sunscreen application technique and of a newly developed systematized application technique (Dose, Apply, Spread) were compared. Evaluation criteria included the amount of product applied, the homogeneity of sunscreen application as measured by the Wood's lamp, and the volunteers' appreciation of the new technique. RESULTS: Fifty-eight volunteers participated in the study: 20 women, 19 men, and 19 children. Respecting the new application technique resulted in a statistically significant (P < 0.05) more evenly spread sunscreen on the different parts of the body and an increase in the amount of product applied. Furthermore, the body surface area covered was significantly increased (P < 0.05), and the new technique was well perceived and accepted by the volunteers. CONCLUSION: The proposed new application technique ensures that more sunscreen will be used and that it will be applied more evenly. Educational work could help improve the efficient use of sunscreens, therefore providing better UV protection.