Sarna noruega: alcoholismo como factor predisponente. Reporte de un caso

Introducción: La escabiosis o sarna humana es una dermatosis causada a partir de la infección cutánea por Sarcoptes scabiei. Una variante severa y rara de ella es la escabiosis noruega o costrosa, que ha repuntado en los últimos años por el incremento en patologías que causan inmunosupresión. Clínicamente se presenta con hiperqueratosis y placas costrosas predominantemente en tórax, cabeza, palmas y plantas. Su diagnóstico es sencillo, mediante la observación microscópica del artrópodo. Se ha descrito su tratamiento con permetrina, benzoato de bencilo e ivermectina. Caso: Paciente masculino de 38 años de edad, con antecedente de sarna noruega tratada hace 2 años y hábito alcohólico acentuado. Inicia enfermedad actual hace 2 meses, caracterizada por pápulas pruriginosas generalizadas que aumentaron progresivamente en cantidad, formando placas costrosas. Examen físico: xerosis cutis, placas hiperqueratósicas de variadas formas y tamaños, algunas coalescentes, fácilmente desprendibles, sobre base eritematosa, brillante, rezumante, localizadas en cuello, tronco y extremidades. Examen microscópico: huevos y adultos de Sarcoptes scabiei. Tratamiento: cefazolina, maleato de clorfeniramina, cetirizina, ivermectina. Discusión: La sarna noruega recurrente en un paciente adulto sin patología de base conocida, debe conllevar a la búsqueda de causas que comprometan la respuesta inmune del individuo. Más allá de las patologías médicas y medicamentos con efectos inmunosupresores conocidos, debe también indagarse el consumo habitual sustancias como alcohol, agente con demostradas propiedades supresoras sobre la respuesta inmune innata y adquirida. En este caso, el tratamiento dermatológico debe ser complementado con el manejo adecuado del hábito alcohólico subyacente a la patología del paciente

Sarna noruega: alcoholismo como factor predisponente. Reporte de un caso

Introducción: La escabiosis o sarna humana es una dermatosis causada a partir de la infección cutánea por Sarcoptes scabiei. Una variante severa y rara de ella es la escabiosis noruega o costrosa, que ha repuntado en los últimos años por el incremento en patologías que causan inmunosupresión. Clínicamente se presenta con hiperqueratosis y placas costrosas predominantemente en tórax, cabeza, palmas y plantas. Su diagnóstico es sencillo, mediante la observación microscópica del artrópodo. Se ha descrito su tratamiento con permetrina, benzoato de bencilo e ivermectina. Caso: Paciente masculino de 38 años de edad, con antecedente de sarna noruega tratada hace 2 años y hábito alcohólico acentuado. Inicia enfermedad actual hace 2 meses, caracterizada por pápulas pruriginosas generalizadas que aumentaron progresivamente en cantidad, formando placas costrosas. Examen físico: xerosis cutis, placas hiperqueratósicas de variadas formas y tamaños, algunas coalescentes, fácilmente desprendibles, sobre base eritematosa, brillante, rezumante, localizadas en cuello, tronco y extremidades. Examen microscópico: huevos y adultos de Sarcoptes scabiei. Tratamiento: cefazolina, maleato de clorfeniramina, cetirizina, ivermectina. Discusión: La sarna noruega recurrente en un paciente adulto sin patología de base conocida, debe conllevar a la búsqueda de causas que comprometan la respuesta inmune del individuo. Más allá de las patologías médicas y medicamentos con efectos inmunosupresores conocidos, debe también indagarse el consumo habitual sustancias como alcohol, agente con demostradas propiedades supresoras sobre la respuesta inmune innata y adquirida. En este caso, el tratamiento dermatológico debe ser complementado con el manejo adecuado del hábito alcohólico subyacente a la patología del paciente

Primary Immune Regulatory Disorders With an Autoimmune Lymphoproliferative Syndrome-Like Phenotype: Immunologic Evaluation, Early Diagnosis and Management

Primary immune regulatory disorders (PIRD) are associated with autoimmunity, autoinflammation and/or dysregulation of lymphocyte homeostasis. Autoimmune lymphoproliferative syndrome (ALPS) is a PIRD due to an apoptotic defect in Fas-FasL pathway and characterized by benign and chronic lymphoproliferation, autoimmunity and increased risk of lymphoma. Clinical manifestations and typical laboratory biomarkers of ALPS have also been found in patients with a gene defect out of the Fas-FasL pathway (ALPS-like disorders). Following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA), we identified more than 600 patients suffering from 24 distinct genetic defects described in the literature with an autoimmune lymphoproliferative phenotype (ALPS-like syndromes) corresponding to phenocopies of primary immunodeficiency (PID) (NRAS, KRAS), susceptibility to EBV (MAGT1, PRKCD, XIAP, SH2D1A, RASGRP1, TNFRSF9), antibody deficiency (PIK3CD gain of function (GOF), PIK3R1 loss of function (LOF), CARD11 GOF), regulatory T-cells defects (CTLA4, LRBA, STAT3 GOF, IL2RA, IL2RB, DEF6), combined immunodeficiencies (ITK, STK4), defects in intrinsic and innate immunity and predisposition to infection (STAT1 GOF, IL12RB1) and autoimmunity/autoinflammation (ADA2, TNFAIP3,TPP2, TET2). CTLA4 and LRBA patients correspond around to 50% of total ALPS-like cases. However, only 100% of CTLA4, PRKCD, TET2 and NRAS/KRAS reported patients had an ALPS-like presentation, while the autoimmunity and lymphoproliferation combination resulted rare n other genetic defects. Recurrent infections, skin lesions, enteropathy and malignancy are the most common clinical manifestations. Some approaches available for the immunological study and identification of ALPS-like patients through flow cytometry and ALPS biomarkers are provided in this work. Protein expression assays for NKG2D, XIAP, SAP, CTLA4 and LRBA deficiencies and functional studies of AKT, STAT1 and STAT3 phosphorylation, are showed as useful tests. Patients suspected to suffer from one of these disorders require rapid and correct diagnosis allowing initiation of tailored specific therapeutic strategies and monitoring thereby improving the prognosis and their quality of life

Linear Strain Tensors on Hyperbolic Surfaces and Asymptotic Theories for Thin Shells

We perform a detailed analysis of the solvability of linear strain equations on hyperbolic surfaces. We prove that if the surface is a smooth noncharacteristic region, any first order infinitesimal isometry can be matched to an infinitesimal isometry of an arbitrarily high order. The implications of this result for the elasticity of thin hyperbolic shells are discussed

Alterations in Circulating Monocytes Predict COVID-19 Severity and Include Chromatin Modifications Still Detectable Six Months after Recovery

This study was supported by the Instituto de Salud Carlos III, Spanish Ministry of Science and Innovation (COVID-19 research call COV20/00181)—co-financed by the European Development Regional Fund “A way to achieve Europe” and by Consejería de Sanidad de la Comunidad de Madrid (CÍVICO study 2020/0082). R.L.G. and O.C.M. hold a research contract “Rio Hortega” (CM19/00120 and CM19/00092, respectively) from the Instituto de Salud Carlos III, Spanish Ministry of Science and Innovation. MCL holds a predoctoral fellowship (FPU19/06393) from the Spanish Ministry of Science and Innovation.An early analysis of circulating monocytes may be critical for predicting COVID-19 course and its sequelae. In 131 untreated, acute COVID-19 patients at emergency room arrival, monocytes showed decreased surface molecule expression, including low HLA-DR, in association with an inflammatory cytokine status and limited anti-SARS-CoV-2-specific T cell response. Most of these alterations had normalized in post-COVID-19 patients 6 months after discharge. Acute COVID-19 monocytes transcriptome showed upregulation of anti-inflammatory tissue repair genes such as BCL6, AREG and IL-10 and increased accessibility of chromatin. Some of these transcriptomic and epigenetic features still remained in post-COVID-19 monocytes. Importantly, a poorer expression of surface molecules and low IRF1 gene transcription in circulating monocytes at admission defined a COVID-19 patient group with impaired SARS-CoV-2-specific T cell response and increased risk of requiring intensive care or dying. An early analysis of monocytes may be useful for COVID-19 patient stratification and for designing innate immunity-focused therapies.Depto. de MedicinaFac. de MedicinaTRUEUnión EuropeaMinisterio de Ciencia e Innovación (España)Comunidad de MadridInstituto de Salud Carlos IIIpu

Sarna noruega: alcoholismo como factor predisponente. Reporte de un caso

La escabiosis o sarna humana es una dermatosis causada a partir de la infección cutánea por Sarcoptes scabiei. Una variante severa y rara de ella es la escabiosis noruega o costrosa, que ha repuntado en los últimos años por el incremento en patologías que causan inmunosupresión. Clínicamente se presenta con hiperqueratosis y placas costrosas predominantemente en tórax, cabeza, palmas y plantas. Su diagnóstico es sencillo, mediante la observación microscópica del artrópodo. Se ha descrito su tratamiento con permetrina, benzoato de bencilo e ivermectina. Caso: Paciente masculino de 38 años de edad, con antecedente de sarna noruega tratada hace 2 años y hábito alcohólico acentuado. Inicia enfermedad actual hace 2 meses, caracterizada por pápulas pruriginosas generalizadas que aumentaron progresivamente en cantidad, formando placas costrosas. Examen físico: xerosis cutis, placas hiperqueratósicas de variadas formas y tamaños, algunas coalescentes, fácilmente desprendibles, sobre base eritematosa, brillante, rezumante, localizadas en cuello, tronco y extremidades. Examen microscópico: huevos y adultos de Sarcoptes scabiei. Tratamiento: cefazolina, maleato de clorfeniramina, cetirizina, ivermectina. Discusión: La sarna noruega recurrente en un paciente adulto sin patología de base conocida, debe conllevar a la búsqueda de causas que comprometan la respuesta inmune del individuo. Más allá de las patologías médicas y medicamentos con efectos inmunosupresores conocidos, debe también indagarse el consumo habitual sustancias como alcohol, agente con demostradas propiedades supresoras sobre la respuesta inmune innata y adquirida. En este caso, el tratamiento dermatológico debe ser complementado con el manejo adecuado del hábito alcohólico subyacente a la patología del paciente.http://revistas.utp.edu.co/index.php/revistamedicaScabies is a dermatosis caused by infection due to Sarcoptes scabiei. An uncommon and severe form of this condition, Norwegian or crusted scabies, has shown an increasing incidence in recent years, due to the growing number of pathologies causing immunosupression. Clinical presentation includes hyperkeratosis and crusted plaques mainly on the skin of the thorax, head, palms and soles. It is easily diagnosed by direct observation of the arhtropode. Permethrin, benzyl benzoate and ivermectin have been described as effective therapeutic resources. Case: A 38- year- old male patient, with previous history of Norwegian scabies treated 2 years ago, and heavy consumption of alcohol is described. Presented illness begins 2 months ago, with the appearance of generalized pruriginous papules which progressively grew into crusted plaques. Physical examination: xerosis cutis, hyperkeratosic plaques in dif erent shapes and sizes, some of them converging, easily removable, on an erytematous, bright, humid base, located on the skin of the neck, torso and limbs. Microscopic examination: eggs and adult forms of Sarcoptes scabiei. Treatment: cefazolin, clorpheniramine, cetirizin, ivermectin. Discussion: Recurrent Norwegian scabies in an adult patient without history of a previous disease, must be a reason to find other causes that jeopardize the individual’s immune response. Besides medical conditions and drugs known to have immunosuppressing effects, habits such as alcohol consumption must be interrogated, for it has been demonstrated that this substance has suppressing effects on innate and acquired immunity. The dermatologic treatment must be complimented with an accurate management of the alcohol abuse which underlies the disease

Predictive autoimmunity using autoantibodies: screening for anti-nuclear antibodies

Background: Early detection of antinuclear antibodies (ANA) in asymptomatic subjects is useful to predict autoimmune diseases years before diagnosis. ANA have been determined by indirect immunofluorescence (IIF) using human epithelial type 2 (HEp-2) cells, which is considered the gold standard technique. Multiplex technology (BioPlex ANA Screen) has been introduced for ANA evaluation in recent years. Nevertheless, concordance between BioPlex and IIF is low and there is no harmonization between both methods for detection of autoantibodies. This study has aimed to clarify the clinical significance of autoantibodies detected by BioPlex ANA Screen in subjects with undiagnosed clinical suspicion of autoimmune disease and to determine the predictive value of autoantibodies detected by BioPlex ANA Screen. Methods: A 3-year follow-up study was performed of 411 subjects without a clear diagnosis of autoimmune diseases in whom autoantibodies were detected by BioPlex ANA Screen that were negative by IIF on HEp-2 cells. Results: At 3 years of follow-up, 312 (76%) subjects were positive for autoantibodies by IIF and 99 subjects continued to be negative. A diagnosis of autoimmune disease was found in most of the subjects (87%). Conclusions: BioPlex ANA Screen has greater sensitivity than IIF on HEp-2 cells for autoantibodies detection. Early detection of these antibodies by BioPlex can predict possible development of autoimmune diseases. Keywords: BioPlex ANA Screen; high sensitivity; indirect immunofluorescence; positive predictive value; predictive autoantibodies; systemic autoimmune rheumatic diseases

Anti-Phospholipid Antibodies and COVID-19 Thrombosis: A Co-Star, Not a Supporting Actor

Background: COVID-19 clinical features include a hypercoagulable state that resembles the antiphospholipid syndrome (APS), a disease characterized by thrombosis and presence of antiphospholipid antibodies (aPL). The relationship between aPL-presence and the appearance of thrombi as well as the transience or permanence of aPL in COVID-19 patients is not sufficiently clear. Methods: A group of 360 COVID-19 patients were followed-up for 6 months. Classic aPL, anti-B2GPI IgA, anti-phosphatidylserine/prothrombin IgG/M and anti-SARS-CoV-2 antibodies were determined at acute phase and >12 weeks later. The reference group included 143 healthy volunteers of the same age-range distribution. Results: aPL prevalence was similar in COVID-19 patients and the reference population. aPL presence in both determinations was significantly associated with thrombosis (OR: 2.33 and 3.71), strong agreement being found for classic aPL and anti-B2GPI IgA (Weighted kappa: 0.85–0.91). Thrombosis-associated aPL occurred a median of 17 days after hospital admission (IQR: 6–28) vs. 4 days for the rest (IQR: 3–7). Although anti-SARS-CoV-2 antibodies levels increased during convalescence, aPL hardly changed. Conclusions: Most COVID-19 patients would carry these aPL before the infection. At least two mechanisms could be behind thrombosis, early immune-dysregulation-mediated thrombosis after infection and belated-aPL-mediated thrombosis, with SARS-CoV-2 behaving as a second hit

Effective Natural Killer Cell Degranulation Is an Essential Key in COVID-19 Evolution

NK degranulation plays an important role in the cytotoxic activity of innate immunity in the clearance of intracellular infections and is an important factor in the outcome of the disease. This work has studied NK degranulation and innate immunological profiles and functionalities in COVID-19 patients and its association with the severity of the disease. A prospective observational study with 99 COVID-19 patients was conducted. Patients were grouped according to hospital requirements and severity. Innate immune cell subpopulations and functionalities were analyzed. The profile and functionality of innate immune cells differ between healthy controls and severe patients; CD56dim NK cells increased and MAIT cells and NK degranulation rates decreased in the COVID-19 subjects. Higher degranulation rates were observed in the non-severe patients and in the healthy controls compared to the severe patients. Benign forms of the disease had a higher granzymeA/granzymeB ratio than complex forms. In a multivariate analysis, the degranulation capacity resulted in a protective factor against severe forms of the disease (OR: 0.86), whereas the permanent expression of NKG2D in NKT cells was an independent risk factor (OR: 3.81; AUC: 0.84). In conclusion, a prompt and efficient degranulation functionality in the early stages of infection could be used as a tool to identify patients who will have a better evolution

Multivariate análisis to APS-events associated-factors.

<p>A. Model with the five most significant variables (selection criteria p<0.01). B. Model replacing the previous model less-significant variable (hypertension) by dyslipidemia. C. Model with the three significant variables in the previous models.</p