RESUMEN
BACKGROUND: Growing evidence shows that atopic dermatitis (AD), food allergy (FA), allergic rhinitis, and asthma are largely determined during the first 1000 days (time elapsed from conception to the 2nd birthday). The ARIES birth cohort aims to determine prenatal and perinatal conditions, as well as genetic and epigenetic factors, that participate in the early setting of immune responses, and the role of these in the later determination of the risk of allergic diseases and asthma in the offspring. METHODS: We have designed a birth cohort of 250 families with prenatal recruitment (~ 14 weeks). We will genotype relevant allergy/asthma-associated variants in trios and will perform immunophenotyping and evaluation of allergy biomarkers in cord blood. At 1 and 2 years of age we will assess if infants have developed allergic sensitization, AD, FA, as well as biomarkers of asthma including the asthma predictive index. We will also evaluate how maternal conditions modify immune programming through epigenetic modifications and will then depict newborn epigenetic cues of allergy/asthma risk. Next, we will assess composition/diversity of maternal gut, placenta, breastmilk and infant gut microbiome and their association with immunophenotype and biomarkers at birth, and clinical outcomes at age 1 and 2. Finally, we plan to assess how environmental exposures (perinatal outdoor and indoor pollution, allergens and endotoxin) affect the incidence of allergic sensitization, AD, FA, and risk of asthma. DISCUSSION: The in-depth study of the ARIES birth cohort shall provide crucial information to understand the rising incidence of allergies and asthma in developing countries, and hopefully provide cues on how to prevent and treat these diseases. TRIAL REGISTRATION: clinicaltrials.gov NCT04186949, retrospectively registered on December 5, 2019.
Asunto(s)
Asma/epidemiología , Dermatitis Atópica/epidemiología , Hipersensibilidad a los Alimentos/epidemiología , Rinitis Alérgica/epidemiología , Asma/etiología , Chile/epidemiología , Dermatitis Atópica/etiología , Femenino , Hipersensibilidad a los Alimentos/etiología , Humanos , Lactante , Recién Nacido , Embarazo , Estudios Prospectivos , Rinitis Alérgica/etiologíaRESUMEN
BACKGROUND: In Chile, there is little access to Dermatology outpatient consultations in the public health care system, which has favored the development of tele dermatology (TD). AIM: To assess satisfaction levels of primary care providers with a TD channel via the social networking app WhatsApp® and the concordance between the diagnosis of general practitioners and dermatologists at Pontificia Universidad Católica de Chile. MATERIAL AND METHODS: An electronic survey was answered by the general practitioners who participate in the TD channel. In 417 cases, the diagnostic concordance between general practitioners and specialists was assessed. RESULTS: The survey was answered by 84 practitioners. General satisfaction levels with the platform were over 95%. Satisfaction levels with the response speed and the management suggested by the specialist were over 90%. Over 80% of the practitioners read the consultation sent by dermatologists and considered that their dermatological knowledge improved. Diagnostic concordance between practitioners and specialists was 41%. CONCLUSIONS: TD via a WhatsApp® group linked to a university is a low cost and easy to implement intervention, generating high levels of satisfaction among general practitioners.
Asunto(s)
Dermatología , Aplicaciones Móviles , Médicos de Atención Primaria , Red Social , Chile , Atención a la Salud , Humanos , Pacientes Ambulatorios , Derivación y ConsultaRESUMEN
BACKGROUND: Stress precipitates mood disorders, characterized by a range of symptoms present in different combinations, suggesting the existence of disease subtypes. Using an animal model, we previously described that repetitive stress via restraint or immobilization induced depressive-like behaviors in rats that were differentially reverted by a serotonin- or noradrenaline-based antidepressant drug, indicating that different neurobiological mechanisms may be involved. The forebrain astrocyte protein aldolase C, contained in small extracellular vesicles, was identified as a potential biomarker in the cerebrospinal fluid; however, its specific origin remains unknown. Here, we propose to investigate whether serum small extracellular vesicles contain a stress-specific protein cargo and whether serum aldolase C has a brain origin. METHODS: We isolated and characterized serum small extracellular vesicles from rats exposed to restraint, immobilization, or no stress, and their proteomes were identified by mass spectrometry. Data available via ProteomeXchange with identifier PXD009085 were validated, in part, by western blot. In utero electroporation was performed to study the direct transfer of recombinant aldolase C-GFP from brain cells to blood small extracellular vesicles. RESULTS: A differential proteome was identified among the experimental groups, including aldolase C, astrocytic glial fibrillary acidic protein, synaptophysin, and reelin. Additionally, we observed that, when expressed in the brain, aldolase C tagged with green fluorescent protein could be recovered in serum small extracellular vesicles. CONCLUSION: The protein cargo of serum small extracellular vesicles constitutes a valuable source of biomarkers of stress-induced diseases, including those characterized by depressive-like behaviors. Brain-to-periphery signaling mediated by a differential molecular cargo of small extracellular vesicles is a novel and challenging mechanism by which the brain might communicate health and disease states to the rest of the body.
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Astrocitos/metabolismo , Moléculas de Adhesión Celular Neuronal/sangre , Proteínas de la Matriz Extracelular/sangre , Vesículas Extracelulares/metabolismo , Fructosa-Bifosfato Aldolasa/sangre , Proteína Ácida Fibrilar de la Glía/sangre , Proteínas del Tejido Nervioso/sangre , Serina Endopeptidasas/sangre , Estrés Psicológico/sangre , Animales , Biomarcadores/sangre , Moléculas de Adhesión Celular Neuronal/genética , Proteínas de la Matriz Extracelular/genética , Vesículas Extracelulares/genética , Fructosa-Bifosfato Aldolasa/genética , Proteína Ácida Fibrilar de la Glía/genética , Masculino , Proteínas del Tejido Nervioso/genética , Mapas de Interacción de Proteínas/fisiología , Ratas , Ratas Sprague-Dawley , Proteína Reelina , Restricción Física/efectos adversos , Restricción Física/psicología , Serina Endopeptidasas/genética , Estrés Psicológico/genética , Estrés Psicológico/psicología , Sinaptofisina/sangre , Sinaptofisina/genéticaRESUMEN
Knuckle pads are benign subcutaneous nodules that appear most frequently on the small joints of the hands. In children, they are often idiopathic, and no universally effective treatment has been reported. We present the case of an adolescent successfully treated with a combination of topical cantharidin -podophylotoxin -salicylic acid.
Asunto(s)
Cantaridina/uso terapéutico , Podofilotoxina/uso terapéutico , Ácido Salicílico/uso terapéutico , Piel/patología , Administración Tópica , Adolescente , Dermoscopía/métodos , Quimioterapia Combinada , Articulaciones de los Dedos/fisiopatología , Estudios de Seguimiento , Dermatosis de la Mano/diagnóstico , Dermatosis de la Mano/tratamiento farmacológico , Humanos , Masculino , Piel/efectos de los fármacos , Resultado del TratamientoRESUMEN
Glomus tumor is a rather common tumor of the hand, which presents with a classic triad of symptoms of high temperature, sensitivity and pain, and localized tenderness. Most glomus tumors are located in the digits, especially in subungueal areas. However, extradigital tumors are less frequent, and little is known about their clinical and histopathological features. Two cases of extradigital symplastic glomus tumor are presented. An active clinical and pathological awareness to avoid misdiagnosis or delayed diagnosis of this uncommon lesion is highlighted.
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Tumor Glómico/patología , Mano , Neoplasias Cutáneas/patología , Adulto , Anciano , Femenino , Tumor Glómico/química , Tumor Glómico/cirugía , Humanos , Neoplasias Cutáneas/química , Neoplasias Cutáneas/cirugíaRESUMEN
Postirradiation pseudosclerodermatous panniculitis presents as an erythematous indurated plaque, on a previously irradiated area. Histopathologically, thick and sclerotic septa and inflammatory infiltrate with foamy histiocytes and adiponecrosis are seen. To date, 12 cases have been reported worldwide. Hereby, we report the case of a 68-year-old woman who developed an indurated painful erythematous plaque on her right leg after radiotherapy for a soft tissue sarcoma, with a septal panniculitis and lobular adiponecrosis on histopathological examination. This is the 13th case reported and the second of extrathoracic location.
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Dermatosis de la Pierna/patología , Paniculitis/patología , Radiodermatitis/patología , Piel/efectos de la radiación , Anciano , Femenino , Humanos , Dermatosis de la Pierna/etiología , Liposarcoma/radioterapia , Paniculitis/etiología , Radiodermatitis/etiología , Radioterapia Adyuvante/efectos adversos , Esclerosis , Piel/patología , Neoplasias de los Tejidos Blandos/radioterapiaRESUMEN
Chronic kidney disease (CKD) is characterized by loss of renal function. The pathological processes involved in the progression of this condition are already known, but the molecular mechanisms have not been completely explained. Recent reports have shown the intrinsic capacity of the kidney to undergo repair after acute injury through the reexpression of repairing proteins (Villanueva S, Cespedes C, Vio CP. Am J Physiol Regul Integr Comp Physiol 290: R861-R870, 2006). Stimulation with basic fibroblast growth factor (bFGF) could accelerate this process. However, it is not known whether bFGF can induce this phenomenon in kidney cells affected by CKD. Our aim was to study the evolution of renal damage in animals with CKD treated with bFGF and to relate the amount of repairing proteins with renal damage progression. Male Sprague-Dawley rats were subjected to 5/6 nephrectomy (NPX) and treated with bFGF (30 µg/kg, NPX+bFGF); a control NPX group was treated with saline (NPX+S). Animals were euthanized 35 days after bFGF administration. Functional effects were assessed based on serum creatinine levels; morphological damage was assessed by the presence of macrophages (ED-1), interstitial α-smooth muscle actin (α-SMA), and interstitial collagen through Sirius red staining. The angiogenic factors VEGF and Tie-2 and the epithelial/tubular factors Ncam, bFGF, Pax-2, bone morphogenic protein-7, Noggin, Lim-1, Wnt-4, and Smads were analyzed. Renal stem cells were evaluated by Oct-4. We observed a significant reduction in serum creatinine levels, ED-1, α-SMA, and Sirius red as well as an important induction of Oct-4, angiogenic factors, and repairing proteins in NPX+bFGF animals compared with NPX+S animals. These results open new perspectives toward reducing damage progression in CKD.
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Factor 2 de Crecimiento de Fibroblastos/uso terapéutico , Riñón/efectos de los fármacos , Insuficiencia Renal Crónica/tratamiento farmacológico , Animales , Proteínas Morfogenéticas Óseas/metabolismo , Creatinina/sangre , Factor 2 de Crecimiento de Fibroblastos/farmacología , Subunidad alfa del Factor 1 Inducible por Hipoxia/metabolismo , Riñón/metabolismo , Riñón/patología , Masculino , Nefrectomía , Ratas , Ratas Sprague-Dawley , Receptor TIE-2/metabolismo , Insuficiencia Renal Crónica/metabolismo , Insuficiencia Renal Crónica/patología , Factor A de Crecimiento Endotelial Vascular/metabolismoRESUMEN
INTRODUCTION: venous leg ulcers are an important problem in public health due to their high prevalence and treatment cost. The gold standard therapy is the compression bandage. Addition of different substances to the compression therapy in order to accelerate ulcer healing has been attempted but none of them has yielded optimal results. OBJECTIVE: to describe the treatment efficacy of venous leg ulcers treated with triple compression bandage and chitosan gel. METHODS: a longitudinal and descriptive study was conducted in a sample of 16 patients with 26 venous leg ulcers that were treated with triple compression bandage plus a chitosan gel every seven days during nine weeks. Ulcer surface was measured during each treatment step. RESULTS: at the end of the treatment period, 89 percent reduction of the ulcer area was found when compared to the initial area at the beginning of the study. This value represents a reduction rate of 1.8 cm2/week. DISCUSSION: the results obtained in ulcer healing were higher than those previously reported when the compression bandage treatment of venous leg ulcers was performed alone. The observed difference might be due to the effect of the chitosan gel.
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Quitosano/administración & dosificación , Vendajes de Compresión , Úlcera Varicosa/patología , Úlcera Varicosa/terapia , Anciano , Anciano de 80 o más Años , Femenino , Estudios de Seguimiento , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Resultado del TratamientoRESUMEN
Reactive perforating collagenosis is a rare perforating dermatosis clinically characterized by intensely pruritic hyperpigmented papules, plaques, and nodules with a central keratotic plug. Histopathology reveals transepidermal elimination of collagen fibers. Its pathophysiology is still under investigation, but the acquired form has been linked to systemic conditions such as diabetes mellitus and chronic kidney disease. However, it has also been described as a paraneoplastic syndrome. The authors present the case of a 65-year-old diabetic patient in which a myeloproliferative neoplasm was suspected.
Asunto(s)
Enfermedades del Colágeno , Diabetes Mellitus , Síndromes Paraneoplásicos , Insuficiencia Renal Crónica , Enfermedades de la Piel , Anciano , Humanos , Síndromes Paraneoplásicos/diagnósticoRESUMEN
H. pylori infection shows an inverse relationship with allergies. Dendritic cells regulate mucosal immune responses including the induction of T regulatory cells which are fundamental in Helicobacter pylori-induced dampening of allergies. In this respect expression of high-affinity IgE receptor (FcεRI) has been associated with a regulatory dendritic cell profile. Therefore we aimed to evaluate possible mechanisms by which H. pylori infection might modify atopy in pediatric patients. Here we show that H. pylori-infected children exhibited both increased expression of FcεRI on peripheral myeloid and plasmacytoid dendritic cells and higher levels of Foxp3 and Latency Associated Peptide on T regulatory cells. Moreover, exposure to H. pylori drove increased FcεRI expression and IL-10 secretion by both pediatric H. pylori-exposed monocyte derived dendritic cells and T cells. Finally, we show a positive correlation between expression of FcεRI in circulating myeloid DCs and total Treg cells, suggesting that in children, H. pylori infection may have a modulating role in atopy, mediated by both altered surface expression of FcεRI on children's DC and an increased T regulatory cell profile.
Asunto(s)
Células Dendríticas/metabolismo , Regulación de la Expresión Génica , Infecciones por Helicobacter/inmunología , Helicobacter pylori/inmunología , Receptores de IgE/metabolismo , Linfocitos T Reguladores/patología , Adolescente , Niño , Femenino , Factores de Transcripción Forkhead/metabolismo , Infecciones por Helicobacter/sangre , Infecciones por Helicobacter/patología , Humanos , Tolerancia Inmunológica , Inmunoglobulina E/sangre , Interleucina-10/metabolismo , Recuento de Linfocitos , Masculino , Linfocitos T Reguladores/metabolismoRESUMEN
The cell adhesion molecule neuroplastin (Np) is a novel candidate to influence human intelligence. Np-deficient mice display complex cognitive deficits and reduced levels of Plasma Membrane Ca2+ ATPases (PMCAs), an essential regulator of the intracellular Ca2+ concentration ([iCa2+]) and neuronal activity. We show abundant expression and conserved cellular and molecular features of Np in glutamatergic neurons in human hippocampal-cortical pathways as characterized for the rodent brain. In Nptn lox/loxEmx1Cre mice, glutamatergic neuron-selective Np ablation resulted in behavioral deficits indicating hippocampal, striatal, and sensorimotor dysfunction paralleled by highly altered activities in hippocampal CA1 area, sensorimotor cortex layers I-III/IV, and the striatal sensorimotor domain detected by single-photon emission computed tomography. Altered hippocampal and cortical activities correlated with reduction of distinct PMCA paralogs in Nptn lox/loxEmx1Cre mice and increased [iCa2+] in cultured mutant neurons. Human and rodent Np enhanced the post-transcriptional expression of and co-localized with PMCA paralogs in the plasma membrane of transfected cells. Our results indicate Np as essential for PMCA expression in glutamatergic neurons allowing proper [iCa2+] regulation and normal circuit activity. Neuron-type-specific Np ablation empowers the investigation of circuit-coded learning and memory and identification of causal mechanisms leading to cognitive deterioration.
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Encéfalo/citología , Encéfalo/metabolismo , Calcio/metabolismo , Glicoproteínas de Membrana/genética , Neuronas/metabolismo , Anciano , Anciano de 80 o más Años , Animales , Biomarcadores , Encéfalo/diagnóstico por imagen , Encéfalo/fisiopatología , Circulación Cerebrovascular , Trastornos del Conocimiento/genética , Trastornos del Conocimiento/metabolismo , Trastornos del Conocimiento/psicología , Expresión Génica , Humanos , Glicoproteínas de Membrana/metabolismo , Ratones , Ratones Noqueados , Persona de Mediana Edad , Transporte de ProteínasRESUMEN
We have studied the effect of low doses of two widely used antidepressants, fluoxetine (Flx) and reboxetine (Rbx), on excitatory synapses of rat brain cortex and hippocampus. After 15 days of Flx treatment (0.67 mg/kg/day), its plasma level was 20.7+/-5.6 ng/ml. Analysis of postsynaptic densities (PSDs) by immunoblotting revealed no changes in the glutamate receptor subunits GluR1, NR1, NR2A/B, mGluR1alpha nor in the neurotrophin receptor p75(NTR). However, the brain-derived neurotrophic factor (BDNF) receptor TrkB decreased by 42.8+/-6%, and remained decreased after 6 weeks of treatment. The BDNF and TrkB content in homogenates of cortex and hippocampus began to rise at 9 and 15 days, respectively, and remained high for up to 6 weeks. Similar results were obtained following chronic Rbx administration at 0.128 mg/kg/day. We propose that BDNF, whose synthesis is increased by antidepressants, and which is in part released at synaptic sites, binds to TrkB in PSDs, leading to the internalization of the BDNF-TrkB complex and, thus, to a decrease of TrkB in the PSDs. This was paralleled by greater levels of phosphorylated (ie activated) TrkB in the light membrane fraction, that contains signaling endosomes. The retrograde transport of endocyted BDNF/TrkB complexes from spines to cell bodies, where it activates the synthesis of more BDNF, is a protracted process, potentially requiring several cycles of TrkB/BDNF complex endocytosis and transport. This positive feedback mechanism may help explain the time-lag between drug administration and its therapeutic effect, that is, the antidepressant drug paradox.
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Antidepresivos/farmacología , Encéfalo/efectos de los fármacos , Fluoxetina/farmacología , Morfolinas/farmacología , Receptor trkB/metabolismo , Sinapsis/efectos de los fármacos , Animales , Antidepresivos/sangre , Western Blotting/métodos , Encéfalo/citología , Factor Neurotrófico Derivado del Encéfalo/metabolismo , Relación Dosis-Respuesta a Droga , Fluoxetina/sangre , Ácido Glutámico/metabolismo , Inmunoprecipitación/métodos , Masculino , Morfolinas/sangre , Fosfotirosina/metabolismo , Ratas , Ratas Sprague-Dawley , Reboxetina , Fracciones Subcelulares/efectos de los fármacos , Factores de Tiempo , Proteínas de Unión al GTP rab5/metabolismoRESUMEN
Abstract Reactive perforating collagenosis is a rare perforating dermatosis clinically characterized by intensely pruritic hyperpigmented papules, plaques, and nodules with a central keratotic plug. Histopathology reveals transepidermal elimination of collagen fibers. Its pathophysiology is still under investigation, but the acquired form has been linked to systemic conditions such as diabetes mellitus and chronic kidney disease. However, it has also been described as a paraneoplastic syndrome. The authors present the case of a 65-year-old diabetic patient in which a myeloproliferative neoplasm was suspected.
Asunto(s)
Humanos , Anciano , Síndromes Paraneoplásicos/diagnóstico , Enfermedades de la Piel , Enfermedades del Colágeno , Diabetes Mellitus , Insuficiencia Renal CrónicaRESUMEN
Background: In Chile, there is little access to Dermatology outpatient consultations in the public health care system, which has favored the development of tele dermatology (TD). Aim: To assess satisfaction levels of primary care providers with a TD channel via the social networking app WhatsApp® and the concordance between the diagnosis of general practitioners and dermatologists at Pontificia Universidad Católica de Chile. Material and Methods: An electronic survey was answered by the general practitioners who participate in the TD channel. In 417 cases, the diagnostic concordance between general practitioners and specialists was assessed. Results: The survey was answered by 84 practitioners. General satisfaction levels with the platform were over 95%. Satisfaction levels with the response speed and the management suggested by the specialist were over 90%. Over 80% of the practitioners read the consultation sent by dermatologists and considered that their dermatological knowledge improved. Diagnostic concordance between practitioners and specialists was 41%. Conclusions: TD via a WhatsApp® group linked to a university is a low cost and easy to implement intervention, generating high levels of satisfaction among general practitioners.
RESUMEN
INTRODUCTION: Aldosterone can induce changes in the expression or activity of Na(+)/H(+) exchanger isoform 1 (NHE-1) in vascular smooth muscle cells. We aimed to clarify whether chronic mineralocorticoid receptor activation exerts an effect on the activity of NHE-1 in the aorta of mineralocorticoid-induced hypertensive rats. METHODS: Uninephrectomized male Sprague-Dawley rats received subcutaneously 10 mg/week of desoxycorticosterone (DOCA) with or without 20 mg/kg of spironolactone, or vehicle alone (n = 20). After four weeks of treatment, the animals were sacrificed; the aorta was excised for subsequent studies, including histological analysis, RT-PCR, Western blot, measurement of NHE-1 activity and vascular contractility in the presence or absence of the selective NHE-1 inhibitor ethyl-isopropyl amiloride (EIPA). RESULTS: Chronic DOCA treatment increased the NHE-1 activity, systolic and diastolic blood pressure, and aortic wall thickness. All these effects were prevented by co-treatment with Spironolactone (p < 0.05). Phenylephrine-induced vascular contractility was significantly reduced in the DOCA group when EIPA was added in the media (p < 0.05). No significant differences in NHE-1 mRNA or protein levels were detected between groups. CONCLUSIONS: Chronic DOCA administration induced functional and morphological alterations in the rat aorta that are partially explained by enhanced NHE-1 activity and prevented by spironolactone. However, we did not observe changes in the NHE-1 transcript or protein levels, suggesting that the effect may be due to post-transcriptional modifications induced by mineralocorticoid receptor activation.
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Aorta/metabolismo , Hipertensión/inducido químicamente , Hipertensión/metabolismo , Intercambiadores de Sodio-Hidrógeno/antagonistas & inhibidores , Espironolactona/farmacología , Animales , Presión Sanguínea/efectos de los fármacos , Desoxicorticosterona/farmacología , Hipertensión/fisiopatología , Masculino , Mineralocorticoides , Fenilefrina/farmacología , Ratas Sprague-Dawley , Intercambiador 1 de Sodio-Hidrógeno , Intercambiadores de Sodio-Hidrógeno/metabolismo , Coloración y Etiquetado , Vasoconstricción/efectos de los fármacosRESUMEN
La mastitis granulomatosa idiopática (IGM, por sus siglas en inglés) es una afección inflamatoria crónica infrecuente y benigna de los senos. Puede simular tres trastornos mamarios muy frecuentes: carcinoma de mama, mastitis y absceso mamario. La IGM se presenta típicamente como una masa mamaria unilateral y dolorosa. La etiología de la IGM no está bien definida, pero se ha propuesto que podría ser una reacción inmune localizada del tejido mamario.El diagnostico de IGM recurrente es complejo porque los hallazgos clínicos y radiológicos no son específicos, por lo que el estudio histopatológico es crucial. El cáncer de mama, la inflamación gra-nulomatosa infecciosa y no infecciosa deben des-cartarse. El tratamiento de la IGM es controver-tido, e incluye vigilancia estrecha, medicamentos inmunosupresores, antibióticos si hay evidencia de infección y escisión quirúrgica. Presentamos un caso de IGM recurrente tratada con dapsona, con buena respuesta a tratamiento, demostrando que este fármaco podría ser una buena alternativa terapéutica debido a su efecto inmunomodulador, antiinflamatorio y ahorrador de esteroides.
Idiopathic granulomatous mastitis (IGM) is an uncommon, non-malignant, chronic inflamma-tory breast condition. It can mimic three very fre-quent breast disorders: breast carcinoma, mastitis and breast abscess. IGM typically presents as a unilateral and painful breast mass. The etiology of IGM is not well defined, but it has been pro-posed that it could be localized immune reaction to breast tissue. The diagnosis of recurrent IGM is complex be-cause clinical and radiological findings are nons-pecific, therefore histopathologic evaluation is crucial. Breast cancer and infectious and nonin-fectious granulomatous inflammation should be discarded. Treatment of IGM is controversial, including close monitoring, immunosuppressive drugs, antibiotics if there is infection evidence and surgical excision. This is a case report of recurrent IGM treated with Dapsone, with good response to treatment, showing that this drug could be a good therapeutic alternative due to its immunomodulatory and anti-inflammatory and steroid sparing.
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Dapsona/uso terapéutico , Fármacos Dermatológicos/uso terapéutico , Mastitis Granulomatosa/tratamiento farmacológico , Recurrencia , Mastitis Granulomatosa/diagnósticoRESUMEN
BACKGROUND: Scleromyxedema is a rare generalized form of lichen myxedematosus, a chronic cutaneous mucinosis of unknown etiology usually associated with a monoclonal gammopathy and underlying systemic disorders. It is characterized by the presence of lichenoid papules and diffuse indurations of the skin. Histologically, mucin deposits are observed in the dermis as variable degrees of fibrosis. Numerous treatment modalities have been reported as producing partial or inconsistent responses associated with significant adverse effects. METHODS: We report an unusual case of scleromyxedema not associated with monoclonal gammopathy in a young patient who was treated with thalidomide. RESULTS: Patient remained stable with maintenance of injuries despite treatment with thalidomide. CONCLUSIONS: Scleromyxedema is a rare presentation for which a defined therapeutic regimen remains to be established. Treatment with thalidomide has proved to be effective in the management of these patients. We suggest that these patients should be followed up with periodic protein electrophoresis with immunofixation for a monoclonal component in blood and urine. As the therapeutic approach to scleromyxedema remains challenging and treatment is based on symptomatic presentation, further clinical studies to substantiate an effective therapeutic regimen with a positive long-term safety and risk profile are required.
Asunto(s)
Inmunosupresores/uso terapéutico , Escleromixedema/sangre , Escleromixedema/tratamiento farmacológico , Talidomida/uso terapéutico , Adolescente , Humanos , Masculino , Paraproteinemias/sangre , Escleromixedema/patologíaRESUMEN
Introducción: La expresión variable en la sintomatología de los trastornos temporomandibulares (TTM) es un desafío para el clínico; así, el origen del dolor y la estructura comprometida no son fáciles de identificar. El disco articular está directamente vinculado a la función mandibular y, en condiciones patológicas, puede verse alterada su posición y consecuentemente su zona de carga. Si bien la presencia de terminaciones nerviosas y los troncos nerviosos potencialmente involucrados en su inervación han sido identificados, la forma en que los nervios se distribuyen al interior del disco no se ha precisado aún. El objetivo de este estudio fue describir la distribución nerviosa del disco articular de la articulación temporomandibular en cadáveres humanos. Material y método: Se realizó disección anatómica de ocho discos articulares de articulaciones temporomandibulares del lado derecho de cadáveres conservados. Los discos articulares fueron sometidos al método de tinción de Sihler y observados bajo lupa estereoscópica y transiluminación. Se realizó fotografía estandarizada y se dividió el disco en 5 zonas (medial, lateral, anterior, posterior, central) caracterizando la distribución nerviosa. Resultados: Se observó presencia de estructuras nerviosas en todas las zonas excepto en la zona central. Las estructuras nerviosas discurren como troncos simples o múltiples en dirección antero-posterior con un número variable de colaterales en las áreas lateral y medial del disco, comunicando la zona retrodiscal con la porción posterior del músculo pterigoideo lateral en todas las muestras. Conclusión: Existe un patrón de inervación común en los discos estudiados. El disco se encuentra inervado en su periferia por troncos nerviosos que presentan un trayecto anteroposterior. La comunicación entre distintas regiones anatómicas por continuidad nerviosa, así como el patrón de inervación del disco, podrían explicar situaciones de dolor local y referido en TTM
Introduction: Variable expression of symptomatology in temporomandibular disorders (TMD) is a challenge for clinician, because source of pain is not easy to identify. Articular disc is directly linked to the mandibular function and in pathological conditions can be altered its position and consequelty its zone of load. The aim of this study was to describe the intradiscal nerve distribution of temporomandibular joint disc in human cadavers. Although the presence of sensitive nerve terminals in the disc and the nerve trunks potentially involved have been identified, the way the nerves are distributed inside disc remains unprecised. Material and method: Anatomical dissection of 8 articular discs of the temporomandibular joints on the right side of preserved cadavers was performed. The articular discs were subjected to the Sihler staining method and then observed under a stereoscopic loupe and transillumination, A standardized photograph was perform by arbitrarily dividing the disk into 5 zones (medial, lateral, anterior, posterior, central) and characterized thebnerve distribution. Results: Presence of nerve structures was observed in all areas except the central zone. Nerve structures run as a single or multiple trunk in anterior-posterior direction with a variable number of collateral in lateral and medial areas of disc, comunicating retrodiscal zone and posterior zone of lateral pterigoid muscle in all samples. Conclusion: It can be identified a common inervation pattern in all discs. The disc is innervated in its periphery by nerve trunks that present an anterioriposterior path. The communication between different anatomical regions by nervous continuity, as well as the innervation pattern of the disc, could explain situations of local pain and referred in TMD