RESUMEN
Calcinosis cutis (CC) is a very rare and poorly characterized finding in systemic lupus erythematosus (SLE).1 In this retrospective study, we present our experience of 10 individuals with SLE who developed CC, describing the epidemiology, diagnosis, and clinical characteristics of this rare entity.
Asunto(s)
Calcinosis , Lupus Eritematoso Sistémico , Enfermedades de la Piel , Calcinosis/diagnóstico , Calcinosis/epidemiología , Calcinosis/etiología , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/epidemiología , Estudios Retrospectivos , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/epidemiología , Enfermedades de la Piel/etiologíaRESUMEN
The Asian population currently constitutes a simple majority of the global population, comprising nearly 60%. The percentage of the US population that identifies as Asian is expected to grow to 41 million by the year 2050, making up an eventual 9% of the US population. As the world and US populations of Asian individuals increase, the demand for dermatologic care from this population will increase, requiring dermatologists to become more familiar with the diagnosis and treatment of Asian-specific skin characteristics and diseases. In this contribution, we review skin conditions specific to or relatively more common in Asian patients to help recognition and management of diseases in an increasing Asian patient population. We discuss prurigo pigmentosa, primary cutaneous plasmacytosis, lipodystrophia centrifugalis abdominalis infantilis, Epstein-Barr viru-positive T- and natural killer-cell lymphoproliferative disorders, acquired bilateral nevus of Ota-like macules, and BehÒ«et disease.
Asunto(s)
Lipodistrofia , Nevo , Prurigo , Neoplasias Cutáneas , Humanos , PielRESUMEN
BACKGROUND: Approximately 50% of patients with subacute cutaneous lupus erythematosus (SCLE) meet criteria for systemic lupus erythematosus (SLE). The Systemic Lupus International Collaborating Clinics (SLICC) developed new SLE criteria to improve the American College of Rheumatology (ACR) criteria but the SLICC criteria have not been evaluated in patients with SCLE. OBJECTIVE: We sought to determine how patients with SCLE/SLE meet the ACR and SLICC criteria to compare the 2 sets of criteria. METHODS: This was a retrospective analysis of 107 patients with SCLE enrolled in a database at the University of Pennsylvania. RESULTS: Patients with SCLE/SLE were more likely than those with only SCLE to have oral ulcers, positive anti-double-stranded DNA antibodies, and positive antinuclear antibody test findings using both sets of criteria. Patients with SCLE/SLE were also more likely to have low complement using the SLICC criteria. There was a statistically insignificant increase in individuals meeting the SLICC criteria. LIMITATIONS: Not all patients received comprehensive laboratory testing. CONCLUSIONS: Most patients with SCLE who formally meet criteria for SLE do so based on the laboratory and mucocutaneous criteria. Neither the ACR nor SLICC criteria distinguish patients with SCLE and major internal disease from patients with SCLE without major internal disease.
Asunto(s)
Anticuerpos Antinucleares/inmunología , Lupus Eritematoso Cutáneo/clasificación , Lupus Eritematoso Sistémico/clasificación , Guías de Práctica Clínica como Asunto/normas , Adulto , Anciano , Estudios de Cohortes , Conducta Cooperativa , Bases de Datos Factuales , Diagnóstico Diferencial , Femenino , Humanos , Lupus Eritematoso Cutáneo/diagnóstico , Lupus Eritematoso Cutáneo/inmunología , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/inmunología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Reumatología/normas , Medición de Riesgo , Sociedades Médicas/normasRESUMEN
A patient in her 60s with systemic lupus erythematosus presented to an outpatient dermatology clinic on multiple occasions, with exacerbations of cutaneous lupus after exposure to surgical lighting during dental procedures. Her photosensitivity to surgical lighting suggests that artificial light sources pose potential triggers of lupus erythematosus in extra photosensitive individuals. This case report summarises those potential triggers and some options to decrease exposure from surgical lighting.
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Equipo Dental/efectos adversos , Iluminación/efectos adversos , Lupus Eritematoso Cutáneo/etiología , Lupus Eritematoso Sistémico/complicaciones , Trastornos por Fotosensibilidad/etiología , Atención Odontológica/efectos adversos , Femenino , Humanos , Persona de Mediana EdadRESUMEN
Patients with long-standing type 1 diabetes (T1D) may exhibit defective glucose counterregulation and impaired hypoglycemia symptom recognition that substantially increase their risk for experiencing severe hypoglycemia. The purpose of this study was to determine whether intrahepatic islet transplantation improves endogenous glucose production (EGP) in response to hypoglycemia in T1D patients experiencing severe hypoglycemia. We studied longitudinally subjects (n = 12) with â¼30 years, disease duration before and 6 months after intrahepatic islet transplantation using stepped hyperinsulinemic-hypoglycemic and paired hyperinsulinemic-euglycemic clamps with infusion of 6,6-(2)H2-glucose and compared the results with those from a nondiabetic control group (n = 8). After islet transplantation, HbA1c was normalized, and time spent while hypoglycemic (<70 mg/dL) was nearly abolished as indicated by continuous glucose monitoring. In response to insulin-induced hypoglycemia, C-peptide (absent before transplant) was appropriately suppressed, glucagon secretion was recovered, and epinephrine secretion was improved after transplantation. Corresponding to these hormonal changes, the EGP response to insulin-induced hypoglycemia, which was previously absent, was normalized after transplantation, with a similar effect seen for autonomic symptoms. Because the ability to increase EGP is ultimately required to circumvent the development of hypoglycemia, these results provide evidence that intrahepatic islet transplantation can restore glucose counterregulation in long-standing T1D and support its consideration as treatment for patients with hypoglycemia unawareness experiencing severe hypoglycemia.
Asunto(s)
Glucemia/fisiología , Diabetes Mellitus Tipo 1/terapia , Glucosa/metabolismo , Trasplante de Islotes Pancreáticos/fisiología , Adulto , Femenino , Técnica de Clampeo de la Glucosa , Humanos , Masculino , Persona de Mediana EdadRESUMEN
The protective effects of pharmacological inhibitors of xanthine oxidoreductase (XOR) have implicated XOR in many inflammatory diseases. Nonetheless, the role played by XOR during inflammation is poorly understood. We previously observed that inhibition of XOR within the inflammatory mononuclear phagocytes (MNP) prevented neutrophil recruitment during adoptive transfer demonstrating the role of XOR in MNP-mediated neutrophil recruitment. To further explore the role of XOR in the inflammatory state of MNP, we studied MNP isolated from inflammatory lungs combined with analyses of MNP cell lines. We demonstrated that XOR activity was increased in inflammatory MNP following insufflation of Th-1 cytokines in vivo and that activity was specifically increased by MNP differentiation. Inhibition of XOR reduced levels of CINC-1 secreted by MNP. Expression of peroxisome proliferator-activated receptor γ (PPARγ) in purified rat lung MNP and MNP cell lines reflected both the presence of PPARγ isoforms and PPARγ SUMOylation, and XOR inhibitors increased levels of SUMO-PPARγ in MNP cell lines. Both ectopic overexpression of XOR cDNA and uric acid supplementation reduced SUMO-PPARγ in MNP cells. Levels of the M2 markers CD36, CD206, and arginase-1 were modulated by uric acid and oxonic acid, whereas siRNA to SUMO-1 or PIAS-1 also reduced arginase-1 in RAW264.7 cells. We also observed that HIF-1α was increased by XOR inhibitors in inflammatory MNP and in MNP cell lines. These data demonstrate that XOR promotes the inflammatory state of MNP through effects on chemokine expression, PPARγ SUMOylation, and HIF-1α and suggest that strategies for inhibiting XOR may be valuable in modulating lung inflammatory disorders.