RESUMO
OBJECTIVE: To evaluate the characteristics of facial medial capillary malformations (CM), which differ from salmon patches by their wider extent, darker color, and incomplete resolution. STUDY DESIGN: Children were prospectively recruited from pediatric dermatology clinics and retrospectively from clinical and photographic databases. RESULTS: From June 2006 to June 2008, 84 children (56 girls; 66.6%) were included. The medial fronto-FCM (FFCM) involved the forehead and glabella (100%), upper eyelids (57.1%), nose (66.6%), philtrum (50.0%), and upper lip (22.6%). Extended forms were observed in 26.2%. A similar FFCM was observed within the family in 27.3% of cases. Outcome data showed complete regression in 10%, incomplete in 71.1%, and unchanging in 18%. An association with an extra facial CM was found 67.8%. Nape and/or occipital CM were associated in 63.8%. A median dorsal CM, mostly lumbosacral, was observed in 13.4%. An associated disease was seen in 33.3%. Neurological anomalies were observed in 9.5% (two cases of developmental delay, two of epilepsy, one of macrocephaly, one of cerebral arteriovenous malformation, one of cutis marmorata telangiectatica congenita, one of "macrocephaly- cutis marmorata telangiectatica congenita," and one of Rubinstein Taybi syndrome). No correlation between the site or the extent of the FFCM and extrafacial vascular or neurological anomaly was found. CONCLUSIONS: This study identifies a specific type of congenital medial FFCM that looks like salmon patch but has a wider median topography, a darker color, with slower and often incomplete resolution. Family cases are often observed. Despite their slow and incomplete regression, the aesthetic consequences are mild.
Assuntos
Capilares/anormalidades , Face/irrigação sanguínea , Pescoço/irrigação sanguínea , Dermatopatias Vasculares/congênito , Criança , Pré-Escolar , Feminino , França/epidemiologia , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Estudos Prospectivos , Estudos Retrospectivos , Dermatopatias Vasculares/diagnóstico , Dermatopatias Vasculares/epidemiologiaRESUMO
BACKGROUND: Livedoid vasculopathy (LV) is a chronic idiopathic disease characterized by painful purpuric macules on lower extremities. Its exact aetiology remains uncertain, but thrombotic and microcirculatory phenomena have been implicated as possible pathogenic factors. OBJECTIVES: To assess prospectively the frequency of thrombophilia and to verify the effectiveness of anticoagulant therapy among LV patients. METHODS: Thirty-four LV patients were tested for prothrombin time, activated partial thromboplastin time, antithrombin activity, protein C and S activity, anticardiolipin antibodies, lupus anticoagulant, prothrombin gene mutation, factor V Leiden mutation, methylenetetrahydrofolate reductase mutation, plasma homocysteine and fibrinogen. Thirteen of these patients were treated with anticoagulant drugs (either warfarin or heparin). RESULTS: Of 34 patients, 18 (52%) presented laboratory abnormalities of procoagulant conditions. Positive treatment response to anticoagulant therapy was observed in 11 patients. Improvement of pain was obtained in 1-3 weeks, an average of 1.8 week. Complete healing of the lesions was observed in about 2.3 months. Remission was sustained even after treatment interruption and lasted an average 7.8 months. No severe adverse effects were noticed. CONCLUSION: The authors suggest all patients with diagnosis of LV to be investigated for thrombophilic status. Anticoagulant drugs were well tolerated and seemed to be effective in treating not only LV symptoms but also its ulcerations.
Assuntos
Anticoagulantes/uso terapêutico , Dermatopatias Vasculares , Trombofilia , Varfarina/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticorpos Anticardiolipina/sangue , Testes de Coagulação Sanguínea , Fator V/genética , Feminino , Fibrinogênio/metabolismo , Heparina/uso terapêutico , Homocisteína/sangue , Humanos , Inibidor de Coagulação do Lúpus/sangue , Masculino , Metilenotetra-Hidrofolato Redutase (NADPH2)/genética , Pessoa de Meia-Idade , Estudos Prospectivos , Proteína C/metabolismo , Proteína S/metabolismo , Protrombina/genética , Dermatopatias Vasculares/tratamento farmacológico , Dermatopatias Vasculares/epidemiologia , Dermatopatias Vasculares/genética , Trombofilia/tratamento farmacológico , Trombofilia/epidemiologia , Trombofilia/genética , Adulto JovemRESUMO
Systemic lupus erythematosus (SLE) is a complex systemic autoimmune disease with various clinical and serological manifestations. Previous studies have shown the association of SLE and anti-Ro antibody with a series of clinical manifestations. We investigated this association in Brazilian patients with SLE. Five hundred and nine consecutive patients who fulfilled the revised American College of Rheumatology criteria for the SLE were enrolled in the study from June to December 2007. All patients were from our Service of Rheumatology, School of Medicine, University of São Paulo, Brazil. Frequencies of a series of laboratorial and clinical manifestations were calculated. Anti-Ro antibody was associated to anti-La antibody, female, and cutaneous vasculitis. In multivariate analysis, patients with anti-Ro antibody has 1.63 (95% CI 1.07-2.50) more risk to develop cutaneous vasculitis than patients without this antibody. Our data have demonstrated that anti-Ro antibody is an independent useful serologic marker for cutaneous vasculitis.
Assuntos
Anticorpos Antinucleares/sangue , Lúpus Eritematoso Sistêmico/imunologia , Dermatopatias Vasculares/imunologia , Adulto , Brasil/epidemiologia , Comorbidade , Feminino , Humanos , Estudos Longitudinais , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/patologia , Masculino , Estudos Retrospectivos , Fatores Sexuais , Dermatopatias Vasculares/epidemiologia , Dermatopatias Vasculares/patologiaRESUMO
El objetivo del presente estudio fue analizar la prevalencia de lesiones mucocutáneas en pacientes adultos con Lupus Eritematoso Sistémico (LES). Se incluyó a 110 pacientes. El 52,7 por ciento de los participantes presentaron manifestaciones cutáneas como presentación inicial de LES. No hubo diferencias estadísticamente significativas entre los pacientes con manifestaciones cutáneas como presentación inicial de la enfermedad y los pacientes sin manifestaciones cutáneas, en relación a la proporción mujeres/hombres, edad de presentación de los síntomas de LES, edad de diagnóstico de LES e intervalo de tiempo entre la aparición de los primeros síntomas y el diagnóstico de la enfermedad. La prevalencia de manifestaciones mucocutáneas fue de 87,2 por ciento. Los hallazgos más frecuentes fueron fotosensibilidad, eritema malar y fenómeno de Raynaud. Un 40 por ciento de los pacientes presentó lesiones específicas agudas, un 1,8 por ciento, lesiones subagudas y un 15,3 por ciento, lesiones crónicas. Un 84,5 por ciento de los pacientes mostró lesiones inespecíficas. En comparación a series extranjeras, registramos una mayor prevalencia de fotosensibilidad y livedo reticularis y una menor prevalencia de lupus cutáneo subagudo, lupus discoide, alopecia, úlceras orales, vasculitis y úlceras cutáneas crónicas.
The purpose of this study is to analyze the prevalence of mucocutaneous lesions in adults with Systemic Lupus Erythematosus (SLE). We enrolled 110 patients. Cutaneous manifestations were present as the first sign of SLE in 52.7 percent of patients. We found no statistical difference between patients with cutaneous manifestations as the initial finding of the disease and patients without cutaneous manifestations, regarding sex, age at initial symptoms of SLE, age at the diagnosis of SLE and period of time between first symptoms and the diagnosis of SLE. Prevalence of cutaneous manifestations was 87.2 percent. The most common findings were photosensitivity, malar erythema and Raynaud s phenomenon. Specific acute lesions were present in 40 percent of the cases, subacute lesions in 1.8 percent and chronic lesions in 15.3 percent. Non specific lesions were present in 84.5 percent of the cases. In the present study we found a higher prevalence of photosensitivity and livedo reticularis, and a lower prevalence of cutaneous subacute lupus, discoid lupus, alopecia, oral ulcers, vasculitis and chronic cutaneous ulcers, compared with international results.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Dermatopatias/epidemiologia , Dermatopatias/etiologia , Lúpus Eritematoso Sistêmico/complicações , Anti-Inflamatórios/uso terapêutico , Imunossupressores/uso terapêutico , Comorbidade , Chile/epidemiologia , Dermatopatias Vasculares/epidemiologia , Doença de Raynaud/epidemiologia , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Prevalência , Pele/patologia , Distribuição por Sexo , Transtornos de Fotossensibilidade/epidemiologiaRESUMO
Vasculitis can range in severity from a self-limited single-organ disorder to a life-threatening disease with the prospect of multiple-organ failure. This condition presents many challenges to the physician, including classification and diagnosis, appropriate laboratory workup, treatment, and the need for careful follow-up. The physician must not only be able to recognize vasculitis but also be able to provide a specific diagnosis (if possible) as well as recognize and treat any underlying etiologic condition. Most diagnostic criteria are based on the size of vessel involvement, which often correlates with specific dermatologic findings. This may allow the dermatologist to provide an initial diagnosis and direct the medical evaluation. This article reviews the classification and diagnosis of cutaneous vasculitic syndromes and current treatment options; it also presents a comprehensive approach to diagnosing and treating the patient with suspected cutaneous vasculitis. (J Am Acad Dermatol 2003;48:311-40.) LEARNING OBJECTIVE: At the completion of this learning activity, participants should be familiar with the classification and clinical features of the various forms of cutaneous vasculitis. They should also have a rational approach to diagnosing and treating a patient with vasculitis.
Assuntos
Feminino , Masculino , Humanos , Dermatopatias Vasculares/diagnóstico , Dermatopatias Vasculares/epidemiologia , Dermatopatias Vasculares/tratamento farmacológico , Fatores de Risco , Incidência , Prognóstico , Quimioterapia Combinada , Vasculite Leucocitoclástica Cutânea/diagnóstico , Vasculite Leucocitoclástica Cutânea/epidemiologia , Vasculite Leucocitoclástica Cutânea/tratamento farmacológico , Vasculite/classificação , Vasculite/diagnóstico , Vasculite/epidemiologia , Vasculite/etiologia , Vasculite/tratamento farmacológico , Índice de Gravidade de DoençaAssuntos
Dermatopatias Vasculares/epidemiologia , Vasculite/epidemiologia , Adulto , Distribuição por Idade , Idoso , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/epidemiologia , Feminino , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/epidemiologia , Humanos , Incidência , Lactente , América Latina/epidemiologia , Masculino , Pessoa de Meia-Idade , Poliarterite Nodosa/diagnóstico , Poliarterite Nodosa/epidemiologia , Fatores de Risco , Síndrome de Sjogren/epidemiologia , Síndrome de Sjogren/etiologia , Dermatopatias Vasculares/diagnóstico , Arterite de Takayasu/diagnóstico , Arterite de Takayasu/epidemiologia , Vasculite/diagnósticoRESUMO
Las lesiones cutáneas en recién nacidos son frecuentes. La mayoría son banales y transitorias; sin embargo, algunas requieren seguimiento o son marcadores de otras enfermedades importantes. El objetivo de este trabajo fue determinar la frecuencia de presentación y algunas características de diferentes lesiones cutáneas en un grupo de 1.203 recién nacidos de hasta 72 horas de vida. De ellos, 91,2 por ciento presentó alguna lesión cutánea clasificada según criterios clínicos como pigmentada, vascular y otros. Los resultados mostraron mancha mongólica en 45 por ciento, nevos pigmentados en 2 por ciento y manchas café con leche en 2 por ciento. La lesión vascular más frecuente fue la mancha salmón (44 por ciento). Otras lesiones cutáneas frecuentes fueron eritema tóxico (42 por ciento) e hiperplasia de glándulas sebáceas (32 por ciento). El reconocimiento adecuado de lesiones cutáneas al momento del nacimiento es muy importante para definir pronóstico y evolución