Síndrome de Wells: presentación con un gran polimorfismo clínico

El síndrome de Wells es una afección dérmica que presenta un gran polimorfismo clínico. Es una patología autolimitada, recurrente y su origen es incierto. Reportamos el caso de una paciente de 29 años, procedente del área Central de Paraguay, comerciante, sin patología de base que consultó por edema de manos y piel. El tratamiento indicado fue corticoide vía oral y desparasitación con buena evolución y sin recidivas.

Wells syndrome is a dermal condition with a high clinical polymorphism. It is a self-limited, recurrent pathology and its origin is uncertain. We report the case of a 29-year-old female patient from the central area of Paraguay, a shopkeeper, with no underlying pathology, who consulted for hand and skin edema. The indicated treatment was oral corticosteroid and deworming with good evolution and no recurrences.

Eosinophilic Pneumonia Induced by Daptomycin.

Daptomycin-induced eosinophilic pneumonia (DIEP) is a rare but serious complication associated with the use of this broad-spectrum antibiotic. We present the case of a teenager with a history of nasopharyngeal cancer who developed DIEP while receiving daptomycin to treat an infection associated with an implanted chamber catheter. Symptoms included recurrent dyspnea and peripheral eosinophilia, with radiological findings consistent with DIEP. The pathophysiology involves an immune response triggered by daptomycin, resulting in eosinophilic pulmonary inflammation. Diagnosis requires a thorough evaluation of medical history, clinical laboratory tests, and radiological findings. The main treatment involves discontinuation of daptomycin and, in severe cases, the use of steroids. It is essential to consider DIEP in patients with respiratory failure and bilateral pulmonary opacities who have used daptomycin and to suspect it in those with blood eosinophilia or in bronchoalveolar lavage.

[Syndromic approach in migrant patients: Fever, diarrhea, anemia, eosinophilia and chronic cough]. / Abordaje sindrómico en el paciente inmigrante: fiebre, diarrea, anemia, eosinofilia y tos crónica.

Migrant patients share the same diseases as natives, but biological or environmental differences may lead to distinct prevalence and manifestations of certain syndromes. Some common conditions in Primary Care stand out, such as fever, diarrhea, anemia, eosinophilia, and chronic cough, where it is important to have a special consideration. Fever may indicate a serious imported illness, and malaria should always be ruled out. Diarrhea is generally of infectious origin, and in most cases, management is outpatient. Anemia may indicate malnutrition or malabsorption, while eosinophilia may indicate a parasitic infection. Lastly, chronic cough may be a sign of tuberculosis, especially in immigrants from endemic areas. Family medicine holds a privileged position for the comprehensive, culturally sensitive, and person-centered approach to these conditions.

Adult respiratory distress syndrome (ARDS) due to omeprazole-induced drug reaction with eosinophilia and systemic symptoms (DRESS): Case report and review of the literature.

Eosinophilia in not an uncommon findings in the intensive care unit (ICU); however, DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) syndrome, which is characterized by a hypersensitivity reaction to drugs and manifests as eosinophilia, systemic involvement and maculopapular erythematous rash 2-6 weeks after exposure to the offending drug, is an exceptional occurrence. We present the first case described in the literature of DRESS syndrome with pulmonary involvement in the form of interstitial pneumonitis and persistent adult respiratory distress syndrome (ARDS) secondary to proton pump inhibitors (PPI). The patient made a good recovery after withdrawal of the offending drug and long-term treatment with systemic corticosteroids. We also present a systematic review of all cases of DRESS with pulmonary involvement in the form of interstitial pneumonitis and cases of PPI-induced DRESS published to date; none of these describe pulmonary involvement.

Dress por litio: reporte de un caso pediátrico / Lithium dress: a pediatric case report / Dress por Lítio: relato de caso pediátrico

La reacción a medicamentos con eosinofilia y síntomas sistémicos denominada DRESS(por sus siglas en inglés, Drug Reaction with Eosinophilia and Systemic Symptoms) hace parte de un amplio espectro denominado toxicodermias. La incidencia exacta no es conocida en niños; sin embargo, en la literatura se ha estimado una tasa de mortalidad que puede llegar a ser tan alta como el 10 %. Presentamos el caso de una paciente adolescente con antecedente personal de trastorno afectivo bipolar (TAB), quien recibía de forma ambulatoria sertralina, quetiapina y trazodona. Por presencia de alucinaciones se adicionó litio al manejo. Diez días después acude al servicio de urgencias por aparición de erupción cutánea y síntomas sistémicos, por lo que se sospechó un cuadro clínico secundario a hipersensibilidad a los medicamentos.

The reaction to drugs with eosinophilia and systemic symptoms called DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) is part of a broad spectrum called toxicodermias. The exact incidence is not known in children; However, a mortality rate that can be as high as 10% has been estimated in the literature. We present the case of a teenage patient with a personal history of bipolar affective disorder (BD), who received sertraline, quetiapine and trazodone on an outpatient basis. Due to the presence of hallucinations, lithium was added to the management. Ten days later she went to the emergency department due to the appearance of a skin rash and systemic symptoms, for which a clinical condition secondary to hypersensitivity to medications was suspected.

A reação a medicamentos com eosinofilia e sintomas sistêmicos denominada DRESS (Por suas siglas em inglês Drug Reaction with Eosinophilia and Systemic Symptoms) faz parte de um amplo espectro denominado toxicodermias. A incidência exata não é conhecida em crianças; No entanto, uma taxa de mortalidade que pode chegar a 10% foi estimada na literatura. Apresentamos o caso de um paciente adolescente com história pessoal de transtorno afetivo bipolar (TAB), que recebeu sertralina, quetiapina e trazodona em regime ambulatorial. Devido à presença de alucinações, foi adicionado lítio ao manejo. Dez dias depois, recorreu ao pronto-socorro devido ao aparecimento de erupção cutânea e sintomas sistêmicos, suspeitando-se de quadro clínico secundário a hipersensibilidade a medicamentos.

[Caso clínico: síndrome de DRESS por hidroxicloroquina].

Background: DRESS syndrome (Drug reaction with eosinophilia and systemic symptoms) is an idiosyncratic reaction characterized by peripheral eosinophilia and systemic symptoms: fever, exanthema, lymphadenopathy, hepatitis, atypical lymphocytes and elevated liver enzymes. The incidence is 1 per 10,000 exposures, mortality 10-20%. Treatment is based on suspension of the suspected drug and steroids. Case report: A 42-year-old male with the following important antecedents. AHF: mother and father with Diabetes Mellitus type 2. APP: Arterial Hypertension, Diabetes Mellitus type 2, and bee sting allergy. Current Condition: He started 8 days after ingestion of hydroxychloroquine for probable SARS-COV-2 infection, with headache, facial and neck edema, desquamative dermatosis on trunk and upper extremities, went to private clinic with torpid evolution sent to third level for increased facial and neck edema, which merited orotracheal intubation, management with intravenous steroids and antihistamines. Labs on admission: Leukocytes 20090, platelets 322 thousand, eosinophilia (5%), elevated liver enzymes and acute kidney injury, fulfilling J-SCAR criteria. The patient was discharged due to adequate evolution with follow-up by Allergy and Clinical Immunology, the patient persists with desquamative lesions after 4 weeks and normalization of laboratory parameters. Conclusions: DRESS is a delayed adverse reaction. It is important the diagnostic presumption and the causal relationship with the drugs due to the high mortality rate.

Antecedentes: El síndrome DRESS (Drug reaction with eosinophilia and systemic symptoms) es una reacción idiosincrática, se caracteriza por eosinofilia perifé- rica y síntomas sistémicos: fiebre, exantema, linfadenopatía, hepatitis, linfocitos atípicos y elevación de enzimas hepáticas. La incidencia es de 1 por cada 10,000 exposiciones, mortalidad de 10 a 20%. El tratamiento se basa en la suspensión del fármaco sospechoso y en la aplicación de esteroides. Reporte de caso: Masculino de 42 años con los siguientes antecedentes de importancia. AHF: madre y padre con Diabetes Mellitus tipo 2. APP: Hipertensión Arterial, Diabetes Mellitus tipo 2, y alergia a picadura de abeja. Padecimiento Actual: Lo inicia posterior a 8 días tras la ingesta de hidroxicloroquina por probable infección por SARS-COV-2, con cefalea, edema facial y de cuello, dermatosis descamativa en tronco y extremidades superiores, acude a clínica particular con evolución tórpida enviado a tercer nivel por aumento de edema facial y cuello, que amerito intubación orotraqueal, manejo con esteroides intravenosos y anti- histamínicos. Laboratorios a su ingreso: Leucocitos 20090, plaquetas 322 mil, eosinofilia (5%), elevación de enzimas hepáticas y lesión renal aguda, cumpliendo criterios J-SCAR. Se egresa por adecuada evolución con seguimiento por Alergia e Inmunología Clínica, el paciente persiste con lesiones descamativas posterior a 4 semanas y normalización de parámetros de laboratorios. Conclusión: DRESS es una reacción adversa retardada. Es importante la presunción diagnóstica y la relación causal con los fármacos por la alta tasa de morta- lidad.

Cistitis eosinofílica en mujer con enfermedad renal crónica

Se presenta caso de una mujer de 70 años, con antecedentes de enfermedad renal crónica sin requerimiento dialítico que ingresa con descompensación aguda y que mejora con sesiones de hemodiálisis. Al examen físico llama la atención hematuria franca por lo que se solicita ecografía de pelvis donde aprecia vejiga distendida con coágulo en su interior de 7,2 cm. La tomografía muestra aparente tumor de pared de vejiga. La uretrocistografía confirma una cistitis crónica eosinofílica. Es manejada con hidroxicina teniendo respuesta favorable cediendo episodios de hematuria. La cistitis eosinofílica es una condición médica rara que se presenta con síntomas urinarios tales como disuria y hematuria. Es más común en niños que en adultos. El método diagnóstico es a través de una biopsia de pared vesical por cistoscopía. El manejo está dirigido a aliviar los síntomas. El interés por el caso se debe a la rareza de esta patología.

We present the case of a 70-year-old woman with a history of chronic kidney disease without dialysis who was admitted with acute decompensation and improved with hemodialysis sessions. On physical examination, frank hematuria was noticeable, therefore a pelvic ultrasound was requested, where a distended bladder with a 7.2 cm clot inside was observed. The tomography showed an apparent bladder wall tumor. Cyst urethrography confirmed chronic eosinophilic cystitis. It was managed with hydroxyzine, having a favorable response, reducing episodes of hematuria. Eosinophilic cystitis is a rare medical condition that presents with urinary symptoms such as dysuria and hematuria. It is more common in children than in adults. The diagnostic method is through a bladder wall biopsy by cystoscopy. Management is aimed at relieving symptoms. The interest in the case is due to the rarity of this pathology.

Clozapine-Induced Eosinophilia: a Case Report.

INTRODUCTION: Clozapine is an atypical antipsychotic drug eligible for treatment-resistant schizophrenia. It frequently represents the best and the only choice in resistant schizophrenia. However, its use is feared by many professionals due to its possible adverse effects, such as eosinophilia. CASE REPORT: We report a case of a young white male suffering from treatment-resistant schizophrenia who rapidly developed eosinophilia after starting clozapine. DISCUSSION: We present a case of a 26-year-old white man diagnosed with schizophrenia with poor clinical response to several antipsychotics owing to which clozapine was started. Psychotic symptoms improved dramatically but a progressively ascendant eosinophilia was reported during serial haematological analyses. The patient remained physically asymptomatic. An exhaustive assessment with ancillary diagnostic tests revealed no cause for eosinophilia. Thus, a diagnosis of clozapine-induced eosinophilia was made. The drug was discontinued and eosinophil count progressively returned to normal but psychotic symptoms worsened. CONCLUSIONS: Clozapine treatment is frequently feared due to its possible side effects and complications, delaying its use in refractory schizophrenia. Also, to our knowledge, there are no specific guidelines on how to manage haematological side effects such as eosinophilia. This is problematic as, in some cases, it may lead to an unnecessary withdrawal of clozapine with a worsening of psychotic symptoms. We present a brief discussion of the recent literature on the subject.

Reação a drogas com eosinofilia e sintomas sistêmicos (DRESS): desafio no diagnóstico e tratamento / Drug reaction with eosinophilia and systemic symptoms (DRESS): a diagnostic and treatment challenge

Introdução: A reação a medicamentos com eosinofilia e sintomas sistêmicos (DRESS) trata-se de uma doença grave, sendo sua gravidade relacionada ao grau de acometimento visceral, e sua taxa de mortalidade de cerca de 10%. Seu diagnóstico é desafiador, e a utilização do escore RegiSCAR como ferramenta facilita a formação deste diagnóstico. Objetivo: Analisar os aspectos clínicos, laboratoriais, evolução e classificação dos casos segundo o RegiSCAR dos pacientes internados no serviço de Alergia e Imunologia do Hospital do Servidor Público Estadual de São Paulo, com o diagnóstico de DRESS. Método: Trata-se de um estudo retrospectivo baseado na análise de prontuários de pacientes atendidos no período entre janeiro de 2006 a janeiro de 2020. Resultados: Neste estudo verificou-se maior prevalência do sexo feminino, e a DRESS acometeu principalmente adultos e idosos, tendo como comorbidades mais frequentes as doenças cardiovasculares. Dos sintomas clínicos, 69,2% dos pacientes apresentava febre, e a alteração laboratorial mais encontrada foi a presença de eosinofilia. A lesão cutânea mais frequente foi o exantema maculopapular, e os medicamentos, os anticonvulsivantes. O tempo prévio de uso do medicamento foi de 2,1 semanas, e todos os pacientes receberam corticoide sistêmico como tratamento principal, e 3 pacientes fizeram uso da imunoglubulina humana como tratamento adicional. A mortalidade foi de 7% na fase aguda, e 14% por causas secundárias. Conclusão: A DRESS é uma síndrome complexa grave e potencialmente fatal, cujo diagnóstico é desafiador. O uso do escore preconizado pelo RegiSCAR demonstrou ser importante auxílio na confirmação do diagnóstico e na diferenciação de outras doenças. A mortalidade encontrada destaca a gravidade da doença. Reconhecer e excluir a droga implicada e iniciar um tratamento precoce permite maior chance de sobrevida para estes pacientes.

Introduction: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a serious disease. Its severity is related to the degree of visceral involvement and its mortality rate is approximately 10%. Diagnosis is a challenge, although RegiSCAR scores can facilitate the process. Objective: To analyze clinical and laboratory data, clinical course, and classify cases according to RegiSCAR scores among patients diagnosed with DRESS who were admitted to the Allergy and Immunology service of the Hospital do Servidor Público Estadual de São Paulo. Method: This retrospective study analyzed the medical records of patients seen between January 2006 and January 2020. Results: There was a higher prevalence of women, with DRESS mainly affecting adults and older adults; cardiovascular diseases were the most frequent comorbidity. The most common clinical symptom was fever (69.2%), while the most common laboratory finding was eosinophilia. The most frequent skin lesion was maculopapular rash, and anticonvulsants were the main prescribed drug class. The drug was used for a mean of 2.1 weeks, and all patients received systemic corticosteroids as the main treatment. Human immunoglobulin was used as an additional treatment in 3 patients. Mortality was 7% in the acute phase and 14% due to secondary causes. Conclusion: DRESS is a severe, complex, and potentially fatal syndrome whose diagnosis is challenging. RegiSCAR scores helped confirm diagnosis and differentiate it from other diseases. The disease's mortality highlights its severity. Recognizing and excluding the implicated drug and initiating early treatment led to a greater chance of survival for these patients.

Clozapine-Induced Eosinophilia: a Case Report

Introduction: Clozapine is an atypical antipsychotic drug eligible for treatment-resistant schizophrenia. It frequently represents the best and the only choice in resistant schizophrenia. However, its use is feared by many professionals due to its possible adverse effects, such as eosinophilia. Case report: We report a case of a young white male suffering from treatment-resistant schizophrenia who rapidly developed eosinophilia after starting clozapine. Discussion: We present a case of a 26-year-old white man diagnosed with schizophrenia with poor clinical response to several antipsychotics owing to which clozapine was started. Psychotic symptoms improved dramatically but a progressively ascendant eosinophilia was reported during serial haematological analyses. The patient remained physically asymptomatic. An exhaustive assessment with ancillary diagnostic tests revealed no cause for eosinophilia. Thus, a diagnosis of clozapine-induced eosinophilia was made. The drug was discontinued and eosinophil count progressively returned to normal but psychotic symptoms worsened. Conclusions: Clozapine treatment is frequently feared due to its possible side effects and complications, delaying its use in refractory schizophrenia. Also, to our knowledge, there are no specific guidelines on how to manage haematological side effects such as eosinophilia. This is problematic as, in some cases, it may lead to an unnecessary withdrawal of clozapine with a worsening of psychotic symptoms. We present a brief discussion of the recent literature on the subject.

Introducción: La clozapina es un fármaco antipsicótico atípico eligible para la esquizofrenia resistente al tratamiento. Con frecuencia representa la mejor y la única opción para la esquizofrenia resistente. Sin embargo, muchos profesionales temen utilizarla por sus posibles efectos adversos, como la eosinofilia. Reporte de caso: Se expone el caso de un joven blanco que sufre esquizofrenia resistente al tratamiento y desarrolló eosinofilia rápidamente tras comenzar el tratamiento con clozapina. Discusión: Varón de 26 años con diagnóstico de esquizofrenia y mala respuesta clínica a varios antipsicóticos, por lo que se inició clozapina. Los síntomas psicóticos mejoraron drásticamente, pero los análisis hematológicos seriados informaron una eosinofilia en ascenso progresivo. El paciente permaneció físicamente asintomático. Una evaluación exhaustiva con pruebas de diagnóstico complementarias no reveló ninguna causa de eosinofilia. Por lo tanto, se diagnosticó eosinofilia inducida por clozapina. Se suspendió el fármaco, el recuento de eosinófilos volvió progresivamente a la normalidad, pero los síntomas psicóticos empeoraron. Conclusiones: A menudo se teme tratar con clozapina por sus posibles efectos secundarios y sus complicaciones, lo cual retrasa su uso en la esquizofrenia refractaria. Además, hasta donde sabemos, no existen pautas específicas sobre cómo tratar los efectos secundarios hematológicos como la eosinofilia. Esto es problemático porque, en algunos casos, puede conducir a suspender innecesariamente la clozapina y que empeoren los síntomas psicóticos. Se presenta una breve discusión de la literatura reciente sobre el tema.

Síndrome de Wells secundario a dimetilfumarato. A propósito de un caso clínico / Dimethyl fumarate induced Wells syndrome. A case report

Introducción: El síndrome de Wells, también conocido como celulitis eosinofílica, es una rara dermatosis con aproximadamente 200 casos descritos en la bibliografía. Aquí presentamos un caso clínico de un paciente con esclerosis múltiple y síndrome de Wells secundario a dimetilfumarato (DMF). Caso clínico: Mujer de 41 años que en julio de 2021 inició el tratamiento con DMF. Una semana más tarde, comenzó con prurito en las extremidades derechas, seguido por la aparición de zonas eritematosas con vesículas. El hemograma mostró elevación del recuento de los eosinófilos hasta 2.000 µL. El estudio anatomopatológico evidenció un infiltrado eosinófilo a nivel de la dermis compatible con síndrome de Wells. La evolución clínica fue favorable, con resolución de las lesiones y normalización de la eosinofilia aproximadamente en cuatro semanas. No fue necesario administrar corticoesteroides. Conclusiones: La eosinofilia es rara en los pacientes con EM tratados con DMF y generalmente no precisa ajuste de dosis. A pesar de que las manifestaciones clínicas de la eosinofilia en estos pacientes sean raras, es importante que el médico reconozca los síntomas. Numerosos neurolépticos pueden causar eosinofilia y síntomas sistémicos; por lo tanto, los facultativos deben conocer los riesgos de la asociación entre DMF y neurolépticos, en particular por la quetiapina, que contiene fumarato.(AU)

Introduction: Wells syndrome, also known as eosinophilic cellulitis, is a rare dermatosis with approximately 200 cases previously described in the literature. Here, we present a case of a patient with multiple sclerosis with Wells syndrome induced by dimethyl fumarate (DMF). Case report: A 41-year-old Caucasian woman was treated with DMF in July 2021. One week later, she experienced itching on her upper and lower right arm, followed by the appearance of erythematous plaques covered with vesicles. The complete blood count showed an increased eosinophil count of up to 2,000 µL. The histological images demonstrated dermal eosinophil infiltration concordant with Wells syndrome. The clinical course was benign, with complete resolution of the lesions and normalization of the eosinophil count within four weeks. Administration of corticosteroids was not necessary. Conclusions: Eosinophilia is rare in patients with multiple sclerosis treated with DMF and usually does not require dosage adjustments. Although clinical manifestations of eosinophilia in these patients are very rare, it is important for practitioners to recognize the symptoms. Many neuroleptic drugs can induce eosinophilia and systemic symptoms; therefore, physicians must be aware of the risks associated with DMF and neuroleptic drugs, particularly for quetiapine, which contains fumarate.(AU)

DRESS, una reacción alérgica no mediada por IgE / DRESS, a Non-IgE Mediated Allergic Reaction

Resumen El síndrome de reacción a medicamentos con eosinofilia y síntomas sistémicos (DRESS, por sus siglas en inglés) es una respuesta de hipersensibilidad multisistémica poco frecuente inducida por uno o varios medicamentos que puede inducir una reacción adversa cutánea grave, la cual es difícil de diagnosticar y pone en peligro la vida del paciente si no es identificada y no se recibe tratamiento. Frecuentemente, se manifiesta como una erupción cutánea amplia, linfadenopatía, signos de afectación de órganos viscerales y alteraciones hematológicas, como leucocitosis, eosinofilia y, en ocasiones, linfocitosis atípica que se presentan de 2 a 8 semanas posterior a la administración del fármaco responsable. Los medicamentos responsables con mayor número de reportes son la fenitoína, la carbamazepina, el alopurinol y el abacavir. Se han identificado algunos alelos específicos del antígeno leucocitario humano (HLA) que se asocian a la hipersensibilidad de estos fármacos. La fisiopatología del síndrome de DRESS aún no se conoce por completo, generalmente se trata de una respuesta de hipersensibilidad mediada por células T, al interactuar con el receptor del complejo principal de histocompatibilidad en individuos con factores de susceptibilidad genética, como ocurre en otros cuadros de reacciones graves secundarias a la ingesta de fármacos. Los criterios del European Registry of Severe Cutaneous Adverse Reactions to Drugs (RegiSCAR) son los más utilizados para su diagnóstico. El síndrome de hipersensibilidad inducido por fármacos (DiHS), el síndrome de Stevens-Johnson (SSJ), la necrólisis epidérmica tóxica (NET), y la pustulosis exantemática generalizada aguda (PEGA) deben considerarse ante cualquier exantema que aparezca posterior a la administración de cualquier fármaco. La terapia incluye la eliminación del agente causal lo antes posible, así como los corticosteroides sistémicos, los cuales son los pilares del tratamiento.. Los agentes ahorradores de esteroides, como la ciclosporina, las inmunoglobulinas intravenosas (IVIGs) y otros agentes inmunosupresores, se han utilizado con éxito para contribuir al tratamiento.

Abstract DRESS (drug reaction syndrome with eosinophilia and systemic symptoms) is a rare drug-induced multisystemic hypersensitivity response that can induce a severe cutaneous adverse reaction that is difficult to diagnose and treat. It frequently manifests as an extensive skin rash, systemic symptoms, lymphadenopathy, visceral organ involvement, and hematological alterations, mainly leukocytosis, eosinophilia, and sometimes atypical lymphocytosis that manifest 2 to 8 weeks after continuous administration of the responsible drug. The most prevalent drugs related with this syndrome are phenytoin, carbamazepine, allopurinol, and abacavir. Some specific human leukocyte antigen (HLA) alleles have been identified that are associated with hypersensitivity to these drugs. The pathophysiology of DRESS syndrome is not yet fully understood; the main hypothesis is a T-cell mediated hypersensitivity response when interacting with the major histocompatibility complex receptor in individuals with genetic susceptibility factors. The criteria of the European Registry of Severe Cutaneous Adverse Reactions to Drugs (RegiSCAR) are the most commonly used for the diagnosis of DRESS syndrome. Drug-induced hypersensitivity syndrome (DiHS), Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and acute generalized exanthematous pustulosis (AGEP) should be considered for any rash that appears following the administration of any drug. Therapy of DRESS includes the elimination of the causative agent as soon as possible, as well as systemic corticosteroids which are the cornerstones of treatment. Steroid-sparing agents such as cyclosporine, intravenous immunoglobulins (IVIGs), and other immunosuppressive agents have been used successfully to contribute to treatment.

Subserous Eosinophilic Colitis: A Case Report in a Private Hospital in Lima, Peru

Eosinophilic colitis is a rare gastrointestinal disease that belongs to the group of so-called primary eosinophilic diseases of the digestive tract. There are three types: mucosa, transmural (muscular), and subserous. We present the case of a 23-year-old male patient with a clinical picture of abdominal pain, nausea, chronic diarrhea, and ascites. Parasitic and other secondary etiologies were ruled out. Upper digestive endoscopy was not helpful. Colonoscopy revealed characteristics of inflammation in the distal ileum and ascending colon, the histological findings of which were consistent with eosinophilic colitis. The study of ascitic fluid was suggestive of eosinophilic ascites. The patient received induction treatment with prednisone 40 mg daily orally; remission was achieved after two weeks, and maintenance therapy based on prednisone was continued with the progressive withdrawal of the dose. Control of the disease was successful.

La colitis eosinofílica es una patología gastrointestinal infrecuente que pertenece al grupo de las denominadas enfermedades primarias eosinofílicas del tracto digestivo. Existen 3 tipos: mucosa, transmural (muscular) y subserosa. Presentamos el caso de un paciente varón, de 23 años de edad, con un cuadro clínico de dolor abdominal, náuseas, diarrea crónica y presentación de ascitis. Se descartan etiologías parasitarias y otras secundarias. La endoscopia digestiva alta no fue contribuidora. Mediante una colonoscopia se observaron características de inflamación en el íleon distal y el colon ascendente, cuyos hallazgos histológicos son compatibles con colitis eosinofílica. El estudio de líquido ascítico es sugestivo de ascitis eosinofílica. El paciente recibió tratamiento de inducción con prednisona a 40 mg diarios por vía oral, se logró la remisión a las 2 semanas y se continuó con terapia de mantenimiento a base de prednisona con retiro progresivo de la dosis. Se logró el control de la enfermedad de manera exitosa.

[Eosinophilic granulomatosis with polyangiitis]. / Granulomatosis eosinofílica con poliangeítis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis characterized by the presence of asthma associated with eosinophilia, eosinophilic infiltration of different organs, and vasculitis of small and medium-sized vessels. Although classified as anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, it occurs in less than half of the patients. The disease is infrequent, typically appearing in patients with asthma and affecting multiple organs such as lung, skin and peripheral nervous system. Treatment has been based on the use of glucocorticoids and immunosuppressants. In recent years, progress has been made in the knowledge of the pathophysiology, in treatment with the inclusion of biologic agents, the classification criteria have been revised and new therapeutic recommendations have been published.

Enfermedad de Kimura en paciente no asiática / Kimura’s disease in a non-asian patient

La enfermedad de Kimura es un trastorno inflamatorio benigno poco frecuente, de etiología desconocida y que afecta principalmente a sujetos de origen asiático. Clínicamente se manifiesta como masas subcutáneas, indoloras, sobre todo en la zona de cabeza y el cuello. Suele acompañarse de linfadenopatías, y aumento de los niveles de eosinófilos e IgE en sangre periférica. Se presenta un caso de enfermedad de Kimura, diagnosticado en una mujer caucásica tras la exéresis-biopsia de una masa mandibular de gran tamaño. Describir esta patología ayudará a incluir la misma en el diagnóstico diferencial de masas, sobre todo a nivel cervico-facial, favoreciendo así su adecuado manejo diagnóstico-terapéutico. (AU)

Kimura’s disease is a rare benign inflammatory disorder of unknown etiology that mainly affects individuals of Asian origin. Clinically it manifests as subcutaneous, painless masses, especially in the head and neck area. It is usually accompanied by lymphadenopathies and an increase in eosinophil and IgE levels in peripheral blood. A case of Kimura’s disease is presented, diagnosed in a Caucasian woman after the excision-biopsy of a large mandibular mass. Describing this pathology will help to include it in the differential diagnosis of masses, especially at the cervico-facial area, thus favoring its adequate diagnostic-therapeutic management. (AU)

Periostin as an important biomarker of inflammatory phenotype T2 in Brazilian asthma patients / Periostina como um importante biomarcador do fenótipo inflamatório T2 em pacientes brasileiros com asma

ABSTRACT Objective: The aim of this study was to assess the laboratory performance of periostin associated with a panel of biomarkers to identify the inflammatory phenotype of Brazilian asthma patients. Methods: We evaluated 103 Brazilian individuals, including 37 asthmatics and 66 nonasthmatic controls. Both groups underwent analyses for serum periostin, eosinophil levels in the peripheral blood, the fraction of exhaled nitric oxide (FeNO), total serum IgE, urinary leukotriene E4, and serum cytokines. Results: Higher levels of periostin (p = 0.005), blood eosinophils (p = 0.012), FeNO (p = 0.001), total IgE (p < 0.001), and IL-6 (p ≤ 0.001) were found in the asthmatic patients than the controls. Biomarker analyses by the ROC curve showed an AUC greater than 65%. Periostin (OR: 12,550; 95% CI: 2,498-63,063) and IL-6 (OR: 7,249; 95% CI: 1,737-30,262) revealed to be suitable asthma inflammation biomarkers. Blood eosinophils, FeNO, total IgE, IL-6, TNF, and IFN-g showed correlations with clinical severity characteristics in asthmatic patients. Periostin showed higher values in T2 asthma (p = 0.006) and TNF in non-T2 asthma (p = 0.029). Conclusion: The panel of biomarkers proposed for the identification of the inflammatory phenotype of asthmatic patients demonstrated good performance. Periostin proved to be an important biomarker for the identification of T2 asthma.

RESUMO Objetivo: O objetivo deste estudo foi de avaliar o desempenho laboratorial da periostina associada a um painel de biomarcadores para identificar o fenótipo inflamatório de pacientes brasileiros com asma. Métodos: Foram avaliados 103 indivíduos brasileiros, incluindo 37 asmáticos e 66 controles não asmáticos. Ambos os grupos foram submetidos a análises de periostina sérica, níveis de eosinófilos no sangue periférico, a fração exalada de óxido nítrico (FeNO), IgE sérica total, leucotrieno E4 urinário e citocinas séricas. Resultados: Maiores níveis de periostina (p = 0,005), eosinófilos periféricos (p = 0,012), FeNO (p = 0,001), IgE total (p < 0,001) e IL-6 (p ≤ 0,001) foram encontrados nos pacientes asmáticos do que nos controles. As análises de biomarcadores pela curva ROC mostraram uma AUC superior a 65%. A periostina (OR: 12.550; IC 95%: 2.498-63.063) e a IL-6 (OR: 7.249; IC 95%: 1.737-30.262) se mostraram biomarcadores adequados da inflamação da asma. Eosinófilos periféricos, FeNO, IgE total, IL-6, TNF e IFN-g apresentaram correlação com características clínicas de gravidade em pacientes asmáticos. A periostina teve valores mais elevados na asma T2 (p = 0,006) e o TNF na asma não T2 (p = 0,029). Conclusão: O painel de biomarcadores proposto para a identificação do fenótipo inflamatório de pacientes asmáticos demonstrou bom desempenho. A periostina provou ser um importante biomarcador para a identificação da asma T2.

Eosinofilia asociada a miopatía y diplopía / Eosinophilia-associated myopathy and diplopia

Patient presents in ER with symptoms and history indicative of infectious disease, with muscle pain and double vison being the main complaints. After several consultations, it is decided to admit the patientto the internal medicine ward, where following clinical investigationfocused on a differential diagnosis of eosinophilia and myopathy. (AU)

Paciente acude al servicio de urgencias con un cuadro compatible conpatología infecciosa, siendo dolor muscular y visión doble las principales quejas. Tras consultar con diferentes servicios, se decide ingresoen el servicio de medicina interna, donde la investigación clínica sefocaliza en un diagnóstico diferencial de eosinofilia y miopatía. (AU)

Association of Peripheral Blood and Cutaneous Eosinophils With Bullous Pemphigoid Disease Severity and Treatment Outcomes / Asociación entre eosinófilos de sangre periférica y cutáneos con la gravedad del penfigoide ampolloso y los resultados terapéuticos

Background and aims A dermal inflammatory infiltrate rich in eosinophils is a prominent histological feature of bullous pemphigoid (BP) and peripheral blood eosinophilia has been documented in 50–60% of BP patients. Nevertheless, the impact of circulating and dermal infiltrate eosinophil levels on BP remains poorly understood. The main objective of this work was to investigate the association of peripheral blood and dermal infiltrate eosinophil levels with clinical and immunological characteristics of the disease. Material and methods Retrospective cohort study including all patients diagnosed with BP between 2011 and 2020. Results The study cohort included 233 patients with BP. The mean baseline peripheral blood eosinophil count was 956.3±408.6×106/L and the mean number of tissue eosinophils at the dermal hot spot area was 30.5±19.0. Patients with disseminated presentation (i.e. BSA>50%) had significantly higher peripheral blood eosinophil counts (P=0.028). Mucosal involvement was significantly associated with lower dermal eosinophil count (P=0.001). Requiring inpatient care and relapsing were significantly associated with high peripheral blood eosinophil count (P=0.025; P=0.020, respectively). Among the 68 patients who experienced a relapse, 31 had peripheral blood eosinophilia (i.e. >500×106/L) at relapse (44.2%). Peripheral blood eosinophil counts at baseline and at relapse were significantly correlated (r=0.82, P<0.001). Conclusion Peripheral blood and cutaneous eosinophils levels may be useful biomarkers for disease activity and treatment outcomes in BP. Monitoring peripheral blood eosinophil counts may allow early detection of relapse (AU)

Antecedentes y objetivos El infiltrado inflamatorio dérmico rico en eosinófilos es una característica histológica destacada de penfigoide ampolloso (PA) y eosinofilia en sangre periférica, que se ha documentado en el 50-60% de los pacientes con esta enfermedad. Sin embargo, el impacto de los niveles de eosinófilos circulantes y en infiltrados dérmicos en el PA sigue sin comprenderse. El objetivo principal de este estudio fue investigar la asociación entre los niveles de eosinófilos en sangre periférica y en infiltrados dérmicos y las características clínicas e inmunológicas de esta enfermedad. Material y métodos Estudio de cohorte retrospectivo que incluyó a todos los pacientes con PA entre 2011 y 2020. Resultados El estudio de cohorte incluyó 233 pacientes con PA. El recuento de eosinófilos basal medio en sangre periférica fue de 956,3 ± 408,6 x106/L y el número medio de eosinófilos tisulares en la zona dérmica clave fue de 30,5 ± 19. Los pacientes con presentación diseminada (es decir, BSA >50%) tuvieron conteos de eosinófilos en sangre periférica significativamente superiores (p = 0,028). El compromiso mucoso estuvo significativamente asociado a un conteo de eosinófilos cutáneo inferior (p = 0,001). La necesidad de cuidados hospitalarios y las recaídas estuvieron significativamente asociadas a conteos de eosinófilos en sangre periférica más elevados (p = 0,025; p = 0,020, respectivamente). Entre los 68 pacientes que experimentaron recidiva, 31 tuvieron eosinofilia en sangre periférica (es decir, > 500 x 106/L) en recaída (44,2%). Los conteos de eosinófilos en sangre periférica basales y en recaída se correlacionaron significativamente (r = 0,82, p < 0,001). Conclusione Los niveles de eosinófilos en sangre periférica y cutáneos pueden constituir biomarcadores útiles para la actividad de la enfermedad y los resultados terapéuticos en el PA. Supervisar los conteos de eosinófilos en sangre periférica puede ayudar a detectar la recidiva tempranamente (AU)

[Artículo traducido] Asociación entre eosinófilos de sangre periférica y cutáneos con la gravedad del penfigoide ampolloso y los resultados terapéuticos / Association of Peripheral Blood and Cutaneous Eosinophils With Bullous Pemphigoid Disease Severity and Treatment Outcomes

Antecedentes y objetivos El infiltrado inflamatorio dérmico rico en eosinófilos es una característica histológica destacada de penfigoide ampolloso (PA) y eosinofilia en sangre periférica, que se ha documentado en el 50-60% de los pacientes con esta enfermedad. Sin embargo, el impacto de los niveles de eosinófilos circulantes y en infiltrados dérmicos en el PA sigue sin comprenderse. El objetivo principal de este estudio fue investigar la asociación entre los niveles de eosinófilos en sangre periférica y en infiltrados dérmicos y las características clínicas e inmunológicas de esta enfermedad. Material y métodos Estudio de cohorte retrospectivo que incluyó a todos los pacientes con PA entre 2011 y 2020. Resultados El estudio de cohorte incluyó 233 pacientes con PA. El recuento de eosinófilos basal medio en sangre periférica fue de 956,3 ± 408,6 x106/L y el número medio de eosinófilos tisulares en la zona dérmica clave fue de 30,5 ± 19. Los pacientes con presentación diseminada (es decir, BSA >50%) tuvieron conteos de eosinófilos en sangre periférica significativamente superiores (p = 0,028). El compromiso mucoso estuvo significativamente asociado a un conteo de eosinófilos cutáneo inferior (p = 0,001). La necesidad de cuidados hospitalarios y las recaídas estuvieron significativamente asociadas a conteos de eosinófilos en sangre periférica más elevados (p = 0,025; p = 0,020, respectivamente). Entre los 68 pacientes que experimentaron recidiva, 31 tuvieron eosinofilia en sangre periférica (es decir, > 500 x 106/L) en recaída (44,2%). Los conteos de eosinófilos en sangre periférica basales y en recaída se correlacionaron significativamente (r = 0,82, p < 0,001). Conclusione Los niveles de eosinófilos en sangre periférica y cutáneos pueden constituir biomarcadores útiles para la actividad de la enfermedad y los resultados terapéuticos en el PA. Supervisar los conteos de eosinófilos en sangre periférica puede ayudar a detectar la recidiva tempranamente (AU)

Background and aims A dermal inflammatory infiltrate rich in eosinophils is a prominent histological feature of bullous pemphigoid (BP) and peripheral blood eosinophilia has been documented in 50–60% of BP patients. Nevertheless, the impact of circulating and dermal infiltrate eosinophil levels on BP remains poorly understood. The main objective of this work was to investigate the association of peripheral blood and dermal infiltrate eosinophil levels with clinical and immunological characteristics of the disease. Material and methods Retrospective cohort study including all patients diagnosed with BP between 2011 and 2020. Results The study cohort included 233 patients with BP. The mean baseline peripheral blood eosinophil count was 956.3±408.6×106/L and the mean number of tissue eosinophils at the dermal hot spot area was 30.5±19.0. Patients with disseminated presentation (i.e. BSA>50%) had significantly higher peripheral blood eosinophil counts (P=0.028). Mucosal involvement was significantly associated with lower dermal eosinophil count (P=0.001). Requiring inpatient care and relapsing were significantly associated with high peripheral blood eosinophil count (P=0.025; P=0.020, respectively). Among the 68 patients who experienced a relapse, 31 had peripheral blood eosinophilia (i.e. >500×106/L) at relapse (44.2%). Peripheral blood eosinophil counts at baseline and at relapse were significantly correlated (r=0.82, P<0.001). Conclusion Peripheral blood and cutaneous eosinophils levels may be useful biomarkers for disease activity and treatment outcomes in BP. Monitoring peripheral blood eosinophil counts may allow early detection of relapse (AU)