RESUMO
OBJECTIVE: To determine the incidence and clinical predictors of invasive bacterial infection (IBI) in well-appearing children who present to the emergency department (ED) with fever and petechiae. DESIGN: A prospective, observational, multicentre study was conducted in 18 hospitals between November 2017 and October 2019. PATIENTS: A total of 688 patients were recruited. MAIN OUTCOME MEASURES: The primary outcome was the presence of IBI. Clinical features and laboratory test results were described and related to the presence of IBI. RESULTS: Ten IBIs were found (1.5%), comprising eight cases of meningococcal disease and two of occult pneumococcal bacteraemia. Median age was 26.2 months (IQR 15.3-51.2). Blood samples were obtained from 575 patients (83.3%). Patients with an IBI had a shorter time from fever to ED visit (13.5 hours vs 24 hours) and between fever and rash onset (3.5 hours vs 24 hours). Values for absolute leucocyte count, total neutrophil count, C reactive protein and procalcitonin were significantly higher in patients with an IBI. Significantly fewer patients with a favourable clinical status while in the observation unit were found to have an IBI (2/408 patients, 0.5%) than when clinical status was unfavourable (3/18, 16.7%). CONCLUSIONS: The incidence of IBI among children with fever and petechial rash is lower than previously reported (1.5%). The time from fever to ED visit and to rash onset was shorter in patients with an IBI. Patients with a favourable clinical course during observation in the ED are at lower risk of IBI.
Assuntos
Infecções Bacterianas , Exantema , Púrpura , Infecções Estreptocócicas , Humanos , Criança , Lactente , Pré-Escolar , Estudos Prospectivos , Infecções Bacterianas/epidemiologia , Febre/etiologia , Febre/microbiologia , Serviço Hospitalar de Emergência , Púrpura/diagnóstico , Púrpura/epidemiologia , Púrpura/etiologia , Exantema/epidemiologia , Exantema/etiologiaRESUMO
BACKGROUND: A retrospective study was conducted in order to investigate and describe the characteristics of Immunoglobulin A vasculitis (IgAV), previously known as Henoch-SchÓ§nlein purpura, in the paediatric population of a community-based healthcare delivery system in the Italian region of Abruzzo. METHODS: This is a population-based retrospective chart review of the diagnosis of IgAV in children ages 0 to 18, admitted to the Department of Paediatrics of Chieti and Pescara between 1 January 2000 and 31 December 2016. All children enrolled presented with clinical symptoms and laboratory findings and met the EULAR/PRINTO/PRES 2008 criteria. RESULTS: Two-hundred-eight children met the criteria for IgAV, with the highest incidence reported among children below 7-years of age. A correlation with recent infections was found in 64% of the cohort; the onset was more frequently during the winter and fall. Purpura had a diffuse distribution in the majority of patients; joint impairment was the second most frequent symptom (43%), whereas the gastrointestinal tract was involved in 28% of patients. CONCLUSIONS: Hereby, we confirm the relative benignity of IgAV in a cohort of Italian children; with regards to renal involvement, we report a better outcome compared to other studies. However, despite the low rate of renal disease, we observed a wide use of corticosteroids, especially for the treatment of persistent purpura.
Assuntos
Vasculite por IgA/epidemiologia , Dor Abdominal/epidemiologia , Adolescente , Distribuição por Idade , Anemia/epidemiologia , Artralgia/epidemiologia , Artrite/epidemiologia , Sedimentação Sanguínea , Proteína C-Reativa/análise , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Glucocorticoides/uso terapêutico , Hospitalização , Humanos , Vasculite por IgA/terapia , Imunossupressores/uso terapêutico , Lactente , Recém-Nascido , Infecções/epidemiologia , Itália/epidemiologia , Nefropatias/epidemiologia , Nefropatias/etiologia , Leucocitose/epidemiologia , Masculino , Púrpura/epidemiologia , Estudos Retrospectivos , Estações do Ano , Distribuição por Sexo , Trombocitose/epidemiologiaRESUMO
Coronavirus disease 2019 (COVID-19), an emergent disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has rapidly spread throughout the globe since its discovery in December 2019. Although first appreciated to cause pneumonia, numerous organ systems are now known to be involved. The objective of this article is to review the broad spectrum of cutaneous manifestations reported in association with SARS-CoV-2 infection. The most commonly reported cutaneous manifestations associated with COVID-19 infection include pernio (chilblain)-like acral lesions, morbilliform (exanthematous) rash, urticaria, vesicular (varicella-like) eruptions, and vaso-occlusive lesions (livedo racemosa, retiform purpura). It is important to consider SARS-CoV-2 infection in the differential diagnosis of a patient presenting with these lesions in the appropriate clinical context, as cutaneous manifestations may be present in otherwise asymptomatic individuals, or present before developing other symptoms of infection. With increased access to diagnostic testing, we are beginning to understand the utility and limitations of currently available assays.
Assuntos
COVID-19/epidemiologia , Dermatopatias Virais/epidemiologia , COVID-19/patologia , Comorbidade , Exantema/epidemiologia , Humanos , Púrpura/epidemiologia , SARS-CoV-2 , Pele/patologia , Dermatopatias Virais/patologia , Urticária/epidemiologiaRESUMO
This multicenter, open-label, phase I study assessed the safety and antitumor activity of acalabrutinib in Japanese patients with relapsed/refractory (r/r) B-cell malignancies. Parts 1 (dose confirmation) and 2 (dose expansion) of this three-part study are reported. Treatment was a single dose of 100 mg acalabrutinib (day 1), followed by a washout period and then twice daily 100 mg acalabrutinib in part 1, or twice daily 100 mg acalabrutinib in part 2. Patients from parts 1 and 2 with r/r chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL), and r/r mantle cell lymphoma (MCL) were assessed as r/r CLL/SLL and r/r MCL cohorts, respectively. Twenty-five patients received treatment (part 1, n = 6). Median age was 71.0 years. Nine (one patient from part 1) and 13 (two patients from part 1) patients were included in the r/r CLL/SLL and r/r MCL cohorts, respectively. Treatment-related adverse events (AEs) occurred in 88% of patients (grade ≥3, 36%); the most common were headache (28%) and purpura (24%), both grade 1/2. No AEs resulted in treatment discontinuation or death. Median duration of treatment was 31, 20, and 7 months for part 1, r/r CLL/SLL cohort, and r/r MCL cohort, respectively. Overall response rate (ORR) was 89% and 62% for the r/r CLL/SLL and r/r MCL cohorts, respectively. The median progression-free survival (PFS) was not reached for the r/r CLL/SLL cohort and was 7 months for the r/r MCL cohort. Acalabrutinib (100 mg twice daily) was generally safe and well-tolerated in adult Japanese patients with B-cell malignancies.
Assuntos
Antineoplásicos/administração & dosagem , Benzamidas/administração & dosagem , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Linfoma de Célula do Manto/tratamento farmacológico , Recidiva Local de Neoplasia/tratamento farmacológico , Pirazinas/administração & dosagem , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos/efeitos adversos , Antineoplásicos/farmacocinética , Benzamidas/efeitos adversos , Benzamidas/farmacocinética , Esquema de Medicação , Feminino , Cefaleia/induzido quimicamente , Cefaleia/epidemiologia , Humanos , Japão , Leucemia Linfocítica Crônica de Células B/sangue , Linfoma de Célula do Manto/sangue , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/sangue , Púrpura/induzido quimicamente , Púrpura/epidemiologia , Pirazinas/efeitos adversos , Pirazinas/farmacocinética , Análise de Sobrevida , Resultado do TratamentoRESUMO
BACKGROUND: Pigmented purpuric dermatoses (PPDs) are a group of chronic-relapsing, inflammatory purpuras without vasculitis that typically involve the lower limbs. Five major types could be distinguished, according to the clinical and histopathologic analysis. The etiopathogenesis is still unknown and multiple factors have been considered. Prognosis seems not to be influenced by the different clinical forms of PPDs; nevertheless, no previous studies have investigated whether it could be influenced by the anatomical distribution of the disease. METHODS: We enrolled 70 consecutive patients, including both adults and children with a clinical and histopathological diagnosis of PPD, to investigate possible correlations between the different types of PPDs, their anatomical distribution and prognosis. RESULTS: We observed that patients, both adults and children, with an uncommon localization of the dermatoses, in particular with a diffuse localization (more than one body area affected), presented more frequently a persistent form of PPDs. CONCLUSIONS: This is the first study that attempts to relate the anatomical extension of PPDs and their evolution over time. According to our analysis, it seems to be a statistical significance for the prognosis only for patients with Schamberg disease and involvement of lower limbs. However, studies on a larger population are needed.
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Ceratose , Transtornos da Pigmentação , Púrpura , Adulto , Criança , Humanos , Lactente , Transtornos da Pigmentação/epidemiologia , Prognóstico , Prurido , Púrpura/epidemiologiaAssuntos
Betacoronavirus , Infecções por Coronavirus/epidemiologia , Exantema/diagnóstico , Pneumonia Viral/epidemiologia , Púrpura/diagnóstico , Pele/patologia , COVID-19 , Comorbidade , Exantema/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Pandemias , Púrpura/epidemiologia , SARS-CoV-2Assuntos
Betacoronavirus , Infecções por Coronavirus/epidemiologia , Transtornos da Pigmentação/epidemiologia , Pneumonia Viral/epidemiologia , Púrpura/epidemiologia , Tonsilite/epidemiologia , Adolescente , COVID-19 , Comorbidade , Feminino , Humanos , Pandemias , Transtornos da Pigmentação/diagnóstico , Púrpura/diagnóstico , SARS-CoV-2 , Tonsilite/diagnósticoRESUMO
This study standardized the methods used in the determination of orofacial injuries in Victorian family violence homicides and informed potential control selection for an analytic study. Dental service contacts with family violence victims may be intervention avenues due to the presence of abusive injuries in the orofacial region. All Victorian family homicides from January 2000-September 2018 were identified by determining the kinship/relationship and grouped by age. A 20% random sample of adult cases, aged 18-64 years was selected. The median number of orofacial injuries in categories of injury mechanisms/age/gender and the nature of abusive orofacial injuries was reported for the sample. Of 357 closed cases of family homicide, 261 were adults aged 18-64 years. Offender information and injury mechanism data was available for all closed cases, enabling case selection. Of a random sample of 50 adults, 8 cases were excluded. After 2006, CT scans and photos were present in 20 (91%) and 19 (86.4%) of 22 cases, respectively. The nature and median number of orofacial injuries showed correlation to the reported injury mechanism. Strengths and limitations of the used methods were assessed. Not all cases were compatible for assessment of orofacial injuries, thus serving as an additional criterion for exclusion in our methodology. Further detailed study of the whole population of adults should be limited to the period 2006-2018 where the data is more complete. The mechanism of injury may influence control selection for analytic studies. We present preliminary evidence of the frequent occurrence of orofacial injuries in family violence homicides.
Assuntos
Violência Doméstica , Traumatismos Faciais/epidemiologia , Medicina Legal/métodos , Homicídio , Traumatismos Dentários/epidemiologia , Adolescente , Adulto , Asfixia/mortalidade , Asfixia/patologia , Contusões/mortalidade , Contusões/patologia , Traumatismos Faciais/diagnóstico por imagem , Traumatismos Faciais/patologia , Feminino , Humanos , Lacerações/mortalidade , Lacerações/patologia , Masculino , Pessoa de Meia-Idade , Lesões do Pescoço/diagnóstico por imagem , Lesões do Pescoço/epidemiologia , Lesões do Pescoço/patologia , Fotografação , Púrpura/diagnóstico por imagem , Púrpura/epidemiologia , Púrpura/patologia , Tomografia Computadorizada por Raios X , Traumatismos Dentários/diagnóstico por imagem , Traumatismos Dentários/patologia , Vitória/epidemiologia , Adulto JovemRESUMO
BACKGROUND: Striae distensae have notoriously been difficult to treat due to their extensive involvement of nonfacial skin. Microneedling with its lack of thermal injury during microneedling treatment renders it a viable treatment option in darker skin tones and nonfacial regions due to the reduced risk of postinflammatory hyperpigmentation. OBJECTIVE: To describe the clinical results and side effects of microneedling in a series of 25 individuals with striae distensae. MATERIALS AND METHODS: Twenty-five consecutive adults (SPT I-V) with striae distensae involving the trunk and extremities were treated using a microneedling device. No additional treatments (topical or intralesional) were applied. Two assessors blinded to treatment protocol rated clinical improvement of striae on a 5-point scale. Side effects were monitored and tabulated. RESULTS: Patients received 1 to 3 consecutive monthly treatments. All striae improved at least 50% after an average of 1.8 treatments, and 28% of patients demonstrated more than 75% clinical improvement. Striae in thicker skin regions (e.g., buttocks/thighs) showed comparable clinical improvement than those in thinner skin areas (e.g., breasts) and did not require additional treatment sessions. Side effects were limited to transient erythema in all skin phototypes. No infections or dyspigmentation were observed. CONCLUSION: The clinical results obtained in this study support the safe and effective treatment of striae distensae with microneedling in light and dark skin tones in various body locations. Standardization of treatment protocols are anticipated with further (ongoing) studies.
Assuntos
Técnicas Cosméticas/instrumentação , Agulhas/efeitos adversos , Estrias de Distensão/terapia , Adulto , Técnicas Cosméticas/efeitos adversos , Eritema/epidemiologia , Eritema/etiologia , Extremidades , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos da Pigmentação/epidemiologia , Transtornos da Pigmentação/etiologia , Púrpura/epidemiologia , Púrpura/etiologia , Tronco , Resultado do TratamentoRESUMO
BACKGROUND: The evaluation of children with purpuric rash and fever (PRF) is controversial. Although many of them have viral infections, on occasion such patients may be infected with Neisseria meningitidis. We described all children aged 0-18 years with PRF in southern Israel during the period 2005 ̶ 2016 and compared their microbiologic, laboratory, clinical and outcome characteristics in relation to various etiologies of this syndrome. METHODS: Data were summarized from electronic patient and microbiology files. Viral diagnoses were made by serology and/or PCR. RESULTS: Sixty-nine children with PRF were admitted; 30 (43.48%), 9 (13.04%) and 30 (43.48%) had a syndrome of bacterial, viral or non-established etiology, respectively. N. meningitidis infection was diagnosed in 16/69 (23.19%) patients and in 16/30 (53.33%) patients with bacterial etiology; 14/30 (46.67%) patients suffered from a non-invasive bacterial disease (9 with Rickettsial disease). Adenovirus and Influenza B (3 and 2 cases, respectively) represented the most frequent etiologic agents among patients with viral etiology. More patients with PRF of bacterial etiology were older, of Bedouin ethnicity, looked ill on admission, had higher rates of meningitis and were treated more frequently with antibiotics compared with patients with non-bacterial PRF. Fatality rates among patients with bacterial, viral and non-established etiology were 5/30 (16.7%), 0% and 2/39 (5.1%). CONCLUSIONS: Although PFR was uncommon, high rates of meningococcal infections were recorded in children with PRF, which was associated with high fatality rates. Rickettsial infections were frequent, emphasizing the need for a high index of suspicion for this disease in endemic geographic areas.
Assuntos
Infecções Bacterianas/epidemiologia , Exantema/epidemiologia , Febre/epidemiologia , Púrpura/epidemiologia , Viroses/epidemiologia , Adolescente , Infecções Bacterianas/tratamento farmacológico , Criança , Criança Hospitalizada , Pré-Escolar , Exantema/tratamento farmacológico , Feminino , Humanos , Lactente , Recém-Nascido , Israel/epidemiologia , Masculino , Púrpura/tratamento farmacológico , Viroses/tratamento farmacológicoRESUMO
OBJECTIVES: Onset of primary SS is usually between 40 and 60 years of age, with severe systemic complications in 15% of cases. We sought to determine whether early-onset disease is related to a specific phenotype and if it is predictive of a poor outcome. METHODS: Biological and clinical data from 393 patients recruited in the ASSESS cohort, a French multicentre prospective cohort, were compared according to age at diagnosis. RESULTS: Fifty-five patients had early-onset disease, defined as age ⩽35 years at diagnosis, and presented a significantly higher frequency of salivary gland enlargement (47.2% vs 33.3%, P = 0.045), adenopathy (25.5% vs 11.8%, P = 0.006), purpura (23.6% vs 9.2%, P = 0.002) and renal involvement (16.4% vs 4.4%, P = 0.003). They had a higher frequency of hypergammaglobulinaemia (60.8% vs 26.6%, P < 0.001), RF positivity (41.5% vs 20.2%, P < 0.001), low C3 level (18.9% vs 9.1%, P = 0.032), low C4 level (54.7% vs 40.2%, P = 0.048) and autoantibodies [84.6% with anti-SSA vs 54.4% (P < 0.001) and 57.7% with anti-SSB vs 29.7% (P < 0.001)]. The change in ESSDAI scores between baseline and the 5-year follow-up was significantly different (P = 0.005) with a trend for worsening in the early-onset group (0.72, P = 0.27) and a significant improvement in the later onset group (-1.27, P < 0.0001). CONCLUSION: Early-onset primary SS is associated with a specific phenotype defined by clinical and biological features known to be predictive factors of severe systemic disease. Interestingly, we showed a different evolution of the ESSDAI score depending on the age at disease onset, patients with early-onset disease tending to worsen over time.
Assuntos
Síndrome de Sjogren/diagnóstico , Adulto , Distribuição por Idade , Fatores Etários , Idade de Início , Idoso , Idoso de 80 Anos ou mais , Autoanticorpos/sangue , Complemento C3/análise , Complemento C4/análise , Seguimentos , França/epidemiologia , Humanos , Hipergamaglobulinemia/epidemiologia , Hipergamaglobulinemia/etiologia , Linfadenopatia/epidemiologia , Linfadenopatia/etiologia , Pessoa de Meia-Idade , Fenótipo , Prognóstico , Estudos Prospectivos , Púrpura/epidemiologia , Púrpura/etiologia , Fator Reumatoide/sangue , Índice de Gravidade de Doença , Síndrome de Sjogren/complicações , Síndrome de Sjogren/epidemiologia , Síndrome de Sjogren/imunologiaRESUMO
BACKGROUND: Anti-nuclear antibodies (ANA), anti-extractable nuclear antigens (ENA) and anti-dsDNA antibodies are often associated with cutaneous lupus erythematosus (CLE), with variable frequency depending on skin subtype. However, specific data based on large case-series on the pathogenetic, diagnostic and prognostic meaning of such autoantibodies are still lacking. OBJECTIVE: To characterize the correlations between CLE subtypes as well as LE-non-specific skin lesions and their autoantibody pattern. METHODS: Epidemiological, clinical and immunopathological data of 619 Italian patients with CLE and LE-non-specific skin lesions were analysed. Differences in age, sex, clinical features and autoantibody profile were evaluated in each LE subgroup. RESULTS: Anti-nuclear antibodies (P < 0.0001), anti-dsDNA (P < 0.0001), ENA (P = 0.001), anti-Sm (P = 0.001), anti-RNP (P = 0.004) and anti-histone (P = 0.005) antibodies were associated with SLE. A strong association between ANA (P < 0.0001) and anti-dsDNA (P < 0.0001) and female gender was also found: positive ANA and positive anti-dsDNA had a higher prevalence among females. Chronic CLE resulted to be negatively associated with ENA (OR = 0.51, P < 0.0001), anti-Ro/SSA (OR = 0.49, P < 0.0001) and anti-dsDNA (OR = 0.37, P < 0.0001). Intermittent CLE resulted to be negatively associated with ENA (OR = 0.50, P = 0.007) and ANA (OR = 0.61, P = 0.025). Subacute CLE resulted to be associated with ENA (OR = 5.19, P < 0.0001), anti-Ro/SSA (OR = 3.83, P < 0.0001), anti-Smith (OR = 2.95, P = 0.004) and anti-RNP (OR = 3.18, P = 0.007). Acute CLE resulted to be strongly associated with anti-dsDNA (OR = 6.0, P < 0.0001) and ANA (OR = 18.1, P < 0.0001). LE-non-specific skin lesions resulted to be significantly associated with systemic involvement. Livedo reticularis was significantly associated with ENA (P = 0.007) and anti-Ro/SSA (P = 0.036). Palpable purpura and periungual telangiectasia were significantly associated with ANA. CONCLUSION: According to our findings, some well-known associations between CLE subtypes and autoantibody profile were confirmed; moreover, specific association between autoantibodies and LE-non-specific skin lesions was highlighted. A strict association between anti-ENA and anti-Ro/SSA antibodies and livedo reticularis, ANA and palpable purpura, and ANA and periungual telangiectasia was evidenced.
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Anticorpos Antinucleares/sangue , Lúpus Eritematoso Cutâneo/sangue , Lúpus Eritematoso Cutâneo/epidemiologia , Doença Aguda , Adulto , Antígenos Nucleares/imunologia , Autoantígenos/imunologia , Doença Crônica , Estudos Transversais , DNA/imunologia , Feminino , Histonas/imunologia , Humanos , Itália/epidemiologia , Livedo Reticular/sangue , Livedo Reticular/epidemiologia , Masculino , Pessoa de Meia-Idade , Púrpura/sangue , Púrpura/epidemiologia , RNA Citoplasmático Pequeno/imunologia , Ribonucleoproteínas/imunologia , Fatores Sexuais , Telangiectasia/sangue , Telangiectasia/epidemiologiaRESUMO
Brown diseases comprise disorders leading to hyperpigmentation in skin and nails. Melasma is an acquired skin disorder that is characterized by brownish macules that typically occur on the face. Schamberg disease, also known as progressive pigmented purpura, is characterized by brown pigmentation with pepper spots on their edges. We summarize the epidemiology, pathogenesis, histologic features, and treatment choices for additional brown diseases, including melasma, pigmented purpuric dermatoses, postinflammatory hyperpigmentation, drug-induced hyperpigmentation, and pigmentations due to systemic or physiologic conditions.
Assuntos
Doenças da Unha/etiologia , Doenças da Unha/terapia , Transtornos da Pigmentação/etiologia , Transtornos da Pigmentação/terapia , Doença de Addison/complicações , Doença de Addison/diagnóstico , Cor , Síndrome de Cushing/complicações , Síndrome de Cushing/diagnóstico , Humanos , Inflamação/complicações , Ceratose Seborreica/epidemiologia , Ceratose Seborreica/etiologia , Ceratose Seborreica/terapia , Melanose/epidemiologia , Melanose/etiologia , Melanose/terapia , Mucosa , Doenças da Unha/diagnóstico , Transtornos da Pigmentação/diagnóstico , Transtornos da Pigmentação/epidemiologia , Púrpura/epidemiologia , Púrpura/etiologia , Úlcera Varicosa/diagnóstico , Úlcera Varicosa/etiologiaRESUMO
The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded. Demographics and pSS characteristics were recorded. The EGM were defined by EULAR-SS disease activity index (ESSDAI). Patients were divided into two groups according to their use or not of HCQ therapy. We evaluated the use of HCQ and its relationship to EGM. HCQ therapy was defined as the continuous use of the drug for at least 3 months. A descriptive analysis of demographics and pSS characteristics was performed. We compared the incidence of EGM between groups defined by HCQ therapy using chi2 test or Fisher's exact test. A total of 221 patients were included (97.3% women), mean age, 55.7 years (SD 14). Mean age at diagnosis, 48.8 years (SD 15); median disease duration, 60 months (IQR 35-84). One hundred and seventy patients (77%) received HCQ. About half of the patients had at least one EGM during the course of the disease, 20% of them developed an EGM before the onset of the sicca syndrome and 26% simultaneously with dryness symptom. Overall, EGM were less frequent in those on HCQ therapy (36.5% vs 63.5%, p < 0.001). Considering each EGM individually, the following manifestations were more frequent in the non-treated group: arthritis (p < 0.001), fatigue (p < 0.001), purpura (p = 0.01), Raynaud phenomenon (p = 0.003), and hypergammaglobulinemia (p = 0.006). Immunosuppressive treatment was indicated on 28 patients (12.7%), 13 of which were receiving also HCQ. The first reason for those treatments was the presence of arthritis in 12/28 patients (42.8%), and the drug used in all the cases was methotrexate. Only three patients required immunosuppressive therapy with cyclophosphamide, due to the presence of glomerulonephritis, vasculitis, and interstitial lung disease. None of the patients received biologic therapy. The lower incidence of EGM was observed in patients on HCQ therapy supports its efficacy in pSS. However, further large scale prospective studies are needed to confirm these findings.
Assuntos
Antirreumáticos/uso terapêutico , Hidroxicloroquina/uso terapêutico , Síndrome de Sjogren/complicações , Síndrome de Sjogren/tratamento farmacológico , Adulto , Fadiga/epidemiologia , Fadiga/etiologia , Feminino , Humanos , Hipergamaglobulinemia/epidemiologia , Hipergamaglobulinemia/etiologia , Incidência , Masculino , Pessoa de Meia-Idade , Púrpura/epidemiologia , Púrpura/etiologia , Doença de Raynaud/epidemiologia , Doença de Raynaud/etiologia , Estudos RetrospectivosRESUMO
BACKGROUND: Palatal petechiae are 95% specific for streptococcal pharyngitis. Despite this, and despite prior research demonstrating that Group A Streptococcus (GAS) is a common antecedent to pediatric acute-onset neuropsychiatric syndrome (PANS) episodes, we anecdotally observed a low rate of documented GAS in patients with PANS and palatal petechiae. This retrospective chart review was conducted to formally report the rate of palatal petechiae and concurrent GAS in a cohort of patients with PANS and investigate other etiologic factors. METHODS: The clinical notes of 112 patients seen at the Stanford PANS Clinic who met PANS research criteria were reviewed for mention of palatal petechiae. The medical records of patients who demonstrated palatal petechiae on physical examination were reviewed for signs of infection, a clinical history of trauma, and laboratory results that could indicate other causes of petechiae. RESULTS: Twenty-three patients had documented palatal petechiae on physical examination (ages 5-16, 13/23 [57%] male). Fifteen patients had a rapid GAS test and GAS culture in the Stanford PANS clinic, all with negative results. Evidence of recent GAS infection was found in 8/23 (32%) patients (elevated GAS titers [n = 6] or documentation of a positive rapid GAS test at another facility [n = 2]), one of whom also had potential herpes simplex virus (HSV) infection. One patient had potential HSV infection and recent palatal trauma. No patients had thrombocytopenia. 14/23 (61%) of patients with palatal petechiae had no discernable cause of petechiae. 10/19 (53%) of patients had antihistone antibodies. CONCLUSIONS: Despite the established relationship between palatal petechiae and GAS, no patient with palatal petechiae in our clinic tested positive for GAS and only 32% had evidence of recent GAS. Most did not have an identifiable cause for the palatal lesions. This finding suggests the potential for alternative causes of palatal petechiae or undetectable GAS in our patient population. The high prevalence of palatal petechiae without GAS infection suggests that the pathogenesis of PANS is multifactorial and may involve disruption or inflammation of the microvasculature. Additional research is needed to further elucidate these findings.
Assuntos
Doenças Autoimunes/epidemiologia , Transtornos do Comportamento Infantil/epidemiologia , Transtornos do Neurodesenvolvimento/epidemiologia , Palato/patologia , Púrpura/epidemiologia , Infecções Estreptocócicas/epidemiologia , Streptococcus pyogenes/isolamento & purificação , Doença Aguda , Adolescente , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/psicologia , Criança , Transtornos do Comportamento Infantil/diagnóstico , Transtornos do Comportamento Infantil/psicologia , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Masculino , Transtornos do Neurodesenvolvimento/diagnóstico , Transtornos do Neurodesenvolvimento/psicologia , Palato/microbiologia , Púrpura/diagnóstico , Púrpura/psicologia , Estudos Retrospectivos , Infecções Estreptocócicas/diagnóstico , Infecções Estreptocócicas/psicologiaRESUMO
INTRODUCTION: Febrile purpura (FP) is suggestive of meningococcal disease, requiring almost always further investigations and a treatment based on broad spectrum antibiotics. This study aimed to determine the incidence of meningococcal infections as well as their associated clinical signs in children with febrile purpura hospitalized in the emergency department. METHODS: We conducted a descriptive, retrospective study in the pediatric emergency department at the Children's Hospital of Casablanca over a period of 3 years. The hospitalized children with FP who had undergone bloodculture, whether or not associated with lumbar puncture, were included in the study. Statistical analysis was performed using SPSS v.16 software. RESULTS: We enrolled 96 children, 49 boys and 47 girls. The average age was 53.3 ± 40.5 months. Mean body temperature was 38.9°C. Meningococcal infection was diagnosed in 35/96 children. The diagnosis of meningococcemia was retained in 22 children, associated with meningitis in four patients. Symptoms and physical signs significantly associated with meningococcal infection included lethargy (p = 0.04), convulsions (p = 0.01) and purpura occurring outside the skin area drained by the superior vena cava (p = 0.01). CONCLUSION: FP occurring outside the skin area drained by the superior vena cava or associated with convulsions is srongly related to meningococcal infection, whose incidence seems to be high among Moroccan children.
Assuntos
Bacteriemia/epidemiologia , Febre/etiologia , Infecções Meningocócicas/epidemiologia , Púrpura/etiologia , Antibacterianos/uso terapêutico , Bacteriemia/diagnóstico , Bacteriemia/tratamento farmacológico , Criança , Pré-Escolar , Serviço Hospitalar de Emergência , Feminino , Febre/epidemiologia , Hospitalização , Humanos , Incidência , Lactente , Masculino , Infecções Meningocócicas/diagnóstico , Infecções Meningocócicas/tratamento farmacológico , Marrocos/epidemiologia , Púrpura/epidemiologia , Estudos Retrospectivos , Convulsões/epidemiologia , Convulsões/etiologia , Punção EspinalRESUMO
Pigmented purpuric dermatoses (PPD) are a group of chronic and relapsing cutaneous disorders characterized by a distinct purpuric rash. The diagnosis is made with clinical and histopathological findings. Dermoscopy has rarely been used in the diagnosis of PPD. The aim of our study is to describe the dermoscopic findings in patients with PPD. Eighteen patients who were clinically and histopathologically diagnosed with PPD were studied prospectively. The type and duration of PPD, associated diseases, and medication history of the patients were noted. Dermoscopic examination was performed in all of the patients. Four of the patients were women and 14 of them were men. 16 (88.8%) of them had Shamberg's disease, 1 of them had lichen aureus, and 1 had purpura annularis telangiectoides. Dermoscopic examination revealed multiple irregular red dots, globules and/or patches and brown-coppery coloration on the background in all of the patients, a network of interconnected brown lines in 8, linear vessels in 9, brown dots in 3, grey dots in 3, twisted red loops in 5, comma-like vessels in 2, and red lacunae in 1 patient. The dermoscopic examination of PPD might improve the accuracy of clinical diagnosis.
Assuntos
Dermoscopia/métodos , Transtornos da Pigmentação/epidemiologia , Transtornos da Pigmentação/patologia , Púrpura/epidemiologia , Púrpura/patologia , Adolescente , Adulto , Distribuição por Idade , Idoso , Estudos de Coortes , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Transtornos da Pigmentação/diagnóstico , Prognóstico , Estudos Prospectivos , Púrpura/diagnóstico , Doenças Raras , Estudos de Amostragem , Índice de Gravidade de Doença , Distribuição por Sexo , Turquia , Adulto JovemRESUMO
OBJECTIVE: To determine the nationwide incidence of non-traumatic subconjunctival hemorrhage (NTSCH) in Taiwan from 2000-2011 and to analyze the risk factors for NTSCH using a case-control analysis. METHODS: This is a population-based cohort administrative database study. Randomly selected 1,000,000 residents from the Taiwan National Health Insurance Research Database in 2000 and followed for 12 years to determine the population incidence of NTSCH. Individuals with the first diagnosis of subconjunctival hemorrhage were identified by the corresponding International Classification of Diseases code (ICD) 372.72. Traumatic subconjunctival hemorrhages (ICD-9 codes 921, 871, 850-854 and 959.01) were excluded. The association of NTSCH with various demographic factors, comorbidities and use of medications was studied by a population based case-control analysis using data of 2008-2011. RESULTS: A total of 67,720 patients with a first-time diagnosis of NTSCH were identified during the 12 years period. The mean annual incidence was 65 per 10,000 individuals (crude incidence) and 60 per 10,000 individuals (age- and sex-standardized incidence). The incidence rate of NTSCH was higher in women than in men, [men-women ratio: 0.80 (95% confidence interval: 0.78-0.81)]. The age-specific incidence decreased from childhood to the group of teenagers, after which it increased to a maximal value of 136.2 per 10,000 people in the age group of 60-69 years. Case-control analysis showed that comorbidities of hypertension, purpura and thrombocytopenia, and the use of aspirin were significantly associated with the risk of NTSCH. CONCLUSIONS: This study indicates that NTSCH is a common eye disease that occurs once in 167 individuals in a general East Asian population per year. It occurs more often in women than in men and the age-specific incidence peaked in the age group of 60-69 years. Hypertension, purpura and thrombocytopenia, and the use of aspirin are the major risk factors for NTSCH.
Assuntos
Doenças da Túnica Conjuntiva/epidemiologia , Hemorragia Ocular/epidemiologia , Hipertensão/epidemiologia , Púrpura/epidemiologia , Trombocitopenia/epidemiologia , Adolescente , Adulto , Idoso , Aspirina/efeitos adversos , Criança , Comorbidade , Túnica Conjuntiva/irrigação sanguínea , Túnica Conjuntiva/patologia , Doenças da Túnica Conjuntiva/induzido quimicamente , Doenças da Túnica Conjuntiva/patologia , Hemorragia Ocular/induzido quimicamente , Hemorragia Ocular/patologia , Feminino , Inquéritos Epidemiológicos , Humanos , Hipertensão/patologia , Incidência , Masculino , Pessoa de Meia-Idade , Inibidores da Agregação Plaquetária/efeitos adversos , Púrpura/patologia , Fatores de Risco , Taiwan/epidemiologia , Trombocitopenia/patologiaRESUMO
IMPORTANCE: Infective endocarditis (IE) is a rare disease with poor prognosis. When IE is suspected, skin examination is mandatory to look for a portal of entry and classic skin lesions to help diagnose and manage the condition. OBJECTIVES: To describe the prevalence of and factors associated with dermatological manifestations in patients with definite IE. DESIGN: Observational, prospective, population-based epidemiological study between January 1 and December 31, 2008. Subsequently, collected dermatological data were subjected to post hoc analysis. SETTING AND PARTICIPANTS: Patients (n = 497) diagnosed in 7 French regions and hospitalized in France for definite IE satisfying modified Duke criteria. MAIN OUTCOMES AND MEASURES: Patient and disease epidemiological information was collected, focusing on the most classic dermatological manifestations of IE (Osler nodes, Janeway lesions, purpura, and conjunctival hemorrhages). Disease outcome was also recorded. RESULTS: Among 497 definite IE cases, 487 had known dermatological status. Of 487 cases, 58 (11.9%) had skin manifestations, including 39 (8.0%) with purpura, 13 (2.7%) with Osler nodes, 8 (1.6%) with Janeway lesions, and 3 (0.6%) with conjunctival hemorrhages (5 patients had 2 skin manifestations). Patients with skin manifestations had a higher rate of IE-related extracardiac complications than patients without skin manifestations, particularly cerebral emboli (32.8% vs 18.4%, P = .01), without increased mortality. Patients with purpura had larger cardiac vegetations (18.1 vs 13.7 mm, P = .01), and Janeway lesions were associated with more extracerebral emboli (75.0% vs 31.8%, P = .02). CONCLUSIONS AND RELEVANCE: Specific skin manifestations of IE are associated with a higher risk of complications and should alert physicians to examine for extracardiac complications, notably with cerebral imaging.
Assuntos
Endocardite/diagnóstico , Endocardite/epidemiologia , Dermatopatias/diagnóstico , Dermatopatias/epidemiologia , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Comorbidade , Doenças da Túnica Conjuntiva , Endocardite Bacteriana/diagnóstico , Endocardite Bacteriana/epidemiologia , Feminino , França , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prevalência , Prognóstico , Estudos Prospectivos , Púrpura/diagnóstico , Púrpura/epidemiologia , Índice de Gravidade de Doença , Distribuição por Sexo , Telangiectasia Hemorrágica Hereditária/diagnóstico , Telangiectasia Hemorrágica Hereditária/epidemiologiaRESUMO
OBJECTIVE: To describe the characteristics of bruising and mode of presentation of children referred to the paediatric child protection team with suspected physical abuse (PA), and the extent to which these differ between the children where abuse was confirmed and those where it was excluded. DESIGN: Cross-sectional study. SETTING AND PATIENTS: 519 children, <6 years, referred to two paediatric child protection teams. MAIN OUTCOME MEASURES: The mode of presentation, number, anatomical distribution, size and appearance of bruises according to whether PA was confirmed or excluded. ORs with 95% CI were calculated where relevant. RESULTS: PA was confirmed in 69% of children; the rate varied from 84% when abuse was witnessed, admitted, alleged or where explanation for injury was absent or implausible, to 50% where there was a concerning history. Significantly more children with PA had bruises (89.4%) than PA-excluded (69.9%) and had significantly more sites affected (p<0.001). The odds of a PA child having bruising to: buttocks/genitalia (OR 10.9 (CI 2.6 to 46), left ear (OR 7.10 (CI 2.2 to 23.4), cheeks (Left (OR 5.20 (CI 2.5 to 10.7), Right OR 2.83 (CI 1.5 to 5.4)), neck (OR 3.77 (CI 1.3 to 10.9), trunk (back (OR 2.85 (CI 1.6 to 5.0) front (OR 4.74 (CI 2.2 to 10.2), front of thighs (OR2.48 (CI 1.4 to 4.5) or upper arms (OR 1.90 (CI 1.1 to 3.2) were significantly greater than in children with PA-excluded. Petechiae, linear or bruises with distinct pattern, bruises in clusters, additional injuries or a child known to social services for previous child abuse concerns were significantly more likely in PA. CONCLUSIONS: Features in the presenting history, the extent and pattern of bruising differed between children with confirmed PA and those where abuse was excluded. These findings can provide a deeper understanding of bruising sustained from PA.