[Severe hypereosinophilia as a paraneoplastic syndrome in a patient with differentiated thyroid cancer] / Hipereosinofilia severa como síndrome paraneoplásico en un paciente con cáncer diferenciado de tiroides.

In solid tumors, hypereosinophilia is a rare phenomenon and is mainly associated with mucin-secreting carcinomas. Thyroid tumors associated with neutrophilia and/or eosinophilia have been described exclusively in patients with anaplastic thyroid cancer. Eosinophilia associated with papillary thyroid cancer is extremely rare and there are very few cases currently described. It has been suggested that three cytokines, namely interleukin-3 (IL-3), interleukin-5 (IL-5), and granulocyte-macrophage colony-stimulating factor (GM-CSF), may act as a peptide potential eosinophilic. To date, only three patients with differentiated thyroid cancer associated with eosinophilia have been reported, two of the papillary type and one of the medullary type. A 48-year-old patient consulted in 2022 due to bilateral cervical lymphadenopathy of 3 years' duration associated with wasting syndrome and hypereosinophilia. PET CT was requested, which showed hypermetabolic focus in the right thyroid lobe and lymph node, lung, bone, and liver metastases; Thyroid ultrasound showing a nodule of high suspicion of malignancy and a conglomerate of lymphadenopathy in the right lobe with positive needle wash for thyroglobulin. Hypereosinophilia was evaluated with initial leukocytosis values of GB 30,310/mm3 (10,608/mm3 of eosinophils) to maximum values of GB 77,090/mm3 (eosinophils 20,814/mm3). It was interpreted as paraneoplastic syndrome and corticosteroid therapy was started at immunosuppressive doses without response. Our observations presented in this article are in line with most studies reflecting that paraneoplastic hypereosinophilia is characterized by more advanced disease and poor prognosis.

En los tumores sólidos la hipereosinofilia es un fenómeno raro y se asocia principalmente con carcinomas secretores de mucina. Los tumores tiroideos asociados a neutrofilia y/o eosinofilia se han descrito exclusivamente en pacientes con cáncer anaplásico de tiroides. La eosinofilia asociada con cáncer papilar de tiroides es extremadamente rara y se encuentran muy pocos casos descriptos actualmente. Se ha sugerido que tres citocinas, a saber, la interleucina-3 (IL-3), la interleucina-5 (IL-5) y el factor estimulante de colonias de granulocitos y macrófagos (GM-CSF), pueden actuar como un péptido eosinofílico potencial. Hasta el momento solo se han reportado tres pacientes con cáncer diferenciado de tiroides asociados a eosinofilia, dos de tipo papilar y uno de tipo medular. Paciente de 48 años consultó en el año 2022 por adenopatías cervicales bilaterales de 3 años de evolución asociado a síndrome consuntivo e hipereosinofilia. Se solicitó PET CT que evidenció foco hipermetabólico en lóbulo tiroideo derecho y metástasis ganglionares, pulmonares, óseas y hepáticas; ecografía tiroidea que evidencia en lóbulo derecho nódulo de alta sospecha de malignidad y conglomerado de adenopatías con lavado de aguja positivo para tiroglobulina. Evaluada la hipereosinofilia con valores iniciales de leucocitosis de GB 30310/mm3 (10608/mm3 de eosinófilos) hasta valores máximos de GB 77090/mm3 (eosinófilos 20814/mm3) se interpretó como síndrome paraneoplásico y se inició corticoterapia en dosis inmunosupresoras sin respuesta. Nuestras observaciones presentadas en este artículo están en línea con la mayoría de los estudios que reflejan que la hipereosinofilia paraneoplásica se caracteriza por una enfermedad más avanzada y un mal pronóstico.

Extracellular sombrero vesicles are hallmarks of eosinophilic cytolytic degranulation in tissue sites of human diseases.

Eosinophil sombrero vesicles are large tubular carriers resident in the cytoplasm of human eosinophils, identifiable by transmission electron microscopy, and important for immune mediator transport. Increased formation of sombrero vesicles occurs in activated eosinophils in vitro and in vivo. In tissue sites of eosinophilic cytolytic inflammation, extracellular eosinophil sombrero vesicles are noted, but their frequency and significance in eosinophil-associated diseases remain unclear. Here, we performed comprehensive quantitative transmission electron microscopy analyses and electron tomography to investigate the numbers, density, integrity, and 3-dimensional structure of eosinophil sombrero vesicles in different biopsy tissues from 5 prototypic eosinophil-associated diseases (eosinophilic chronic rhinosinusitis/nasal sinuses, ulcerative colitis/intestines, hypereosinophilic syndrome/skin, dermatitis/skin, and schistosomiasis/rectum). The morphology of extracellular eosinophil sombrero vesicles was also compared with that of cytoplasmic eosinophil sombrero vesicles, isolated by subcellular fractionation from peripheral blood eosinophils. We demonstrated that (i) eosinophil cytolysis, releasing intact sombrero vesicles and membrane-bound granules, is a consistent event in all eosinophil-associated diseases; (ii) eosinophil sombrero vesicles persist intact even after complete disintegration of all cell organelles, except granules (late cytolysis); (iii) the eosinophil sombrero vesicle population, composed of elongated, curved, and typical sombreros, and the eosinophil sombrero vesicle 3-dimensional architecture, diameter, and density remain unchanged in the extracellular matrix; (iv) free eosinophil sombrero vesicles closely associate with extracellular granules; and (v) free eosinophil sombrero vesicles also associate with externalized chromatin during eosinophil ETosis. Remarkably, eosinophil sombrero vesicles appeared on the surface of other cells, such as plasma cells. Thus, eosinophil cytolysis/ETosis can secrete intact sombrero vesicles, alongside granules, in inflamed tissues of eosinophil-associated diseases, potentially serving as propagators of eosinophil immune responses after cell death.

In Vivo ETosis of Human Eosinophils: The Ultrastructural Signature Captured by TEM in Eosinophilic Diseases.

Eosinophilic diseases, also termed eosinophil-associated diseases (EADs), are characterized by eosinophil-rich inflammatory infiltrates and extensive eosinophil degranulation with clinically relevant organ pathology. Recent evidence shows that eosinophil cytolytic degranulation, that is, the release of intact, membrane-delimited granules that arises from the eosinophil cytolysis, occurs mainly through ETosis, meaning death with a cytolytic profile and extrusion of nucleus-originated DNA extracellular traps (ETs). The ultrastructural features of eosinophil ETosis (EETosis) have been studied mostly in vitro after stimulation, but are still poorly understood in vivo. Here, we investigated in detail, by transmission electron microscopy (TEM), the ultrastructure of EETosis in selected human EADs affecting several tissues and organ systems. Biopsies of patients diagnosed with eosinophilic chronic rhinosinusitis/ECRS (frontal sinus), ulcerative colitis/UC (intestine), and hypereosinophilic syndrome/HES (skin) were processed for conventional TEM. First, we found that a large proportion of tissue-infiltrated eosinophils in all diseases (~45-65% of all eosinophils) were undergoing cytolysis with release of free extracellular granules (FEGs). Second, we compared the morphology of tissue inflammatory eosinophils with that shown by in vitro ETosis-stimulated eosinophils. By applying single-cell imaging analysis, we sought typical early and late EETosis events: chromatin decondensation; nuclear delobulation and rounding; expanded nuclear area; nuclear envelope alterations and disruption; and extracellular decondensed chromatin spread as ETs. We detected that 53% (ECRS), 37% (UC), and 82% (HES) of all tissue cytolytic eosinophils had ultrastructural features of ETosis in different degrees. Eosinophils in early ETosis significantly increased their nuclear area compared to non-cytolytic eosinophils due to excessive chromatin decondensation and expansion observed before nuclear envelope disruption. ETosis led not only to the deposition of intact granules, but also to the release of eosinophil sombrero vesicles (EoSVs) and Charcot-Leyden crystals (CLCs). Free intact EoSVs and CLCs were associated with FEGs and extracellular DNA nets. Interestingly, not all cytolytic eosinophils in the same microenvironment exhibited ultrastructure of ETosis, thus indicating that different populations of eosinophils might be selectively activated into this pathway. Altogether, our findings captured an ultrastructural signature of EETosis in vivo in prototypic EADs highlighting the importance of this event as a form of eosinophil degranulation and release of inflammatory markers (EoSVs and CLCs).

Guia prático de urticária aguda / Practical guide to acute urticaria

A urticária aguda é uma causa frequente de consulta com alergistas, caracterizada por urticas e/ou angioedema. Embora autolimitada e benigna, pode causar desconforto significativo e raramente representar uma doença sistêmica grave ou reação alérgica com risco de vida. Nesta revisão, elaborada pelo Departamento Científico de Urticária da Associação Brasileira de Alergia e Imunologia, foram abordadas as principais questões referentes ao tema para auxiliar o médico especialista e generalista.

Acute urticaria is a frequent cause of consultations with allergists, being characterized by wheals and/or angioedema. Although self-limited and benign, it may cause significant discomfort and uncommonly represent a serious systemic disease or life-threatening allergic reaction. In this review prepared by the Urticaria Scientific Department of the Brazilian Association of Allergy and Immunology, the main questions about this topic are addressed to help specialists and general practitioners.

Mepolizumab in Hypereosinophilic Syndrome: A Systematic Review and Meta-analysis.

We aimed to evaluate the efficacy and safety of mepolizumab (MEP) in the management of hypereosinophilic syndrome (HES). A systematic search was performed, and articles published until March 2021 were analyzed. The primary efficacy results evaluated were hospitalization rate related to HES, morbidity (new or worsening), relapses/failure, treatment-related adverse effects, prednisone dosage ≤10 mg/day for ≥8 weeks, and eosinophil count <600/µL for ≥8 weeks. A meta-analysis was conducted, when appropriate. Three randomized controlled trials (RCTs), with a total of 255 patients, were included. The studies contemplated the use of MEP 300 mg/SC or 750 mg/IV. According to the evaluation of the proposed outcomes, when relapse rates/therapeutic failures were assessed, there was a 26% reduction with MEP 300 mg/SC (RD=-0.26; 95% CI: -0.44 to -0.08; p=0.04) and 48% reduction with MEP 750 mg/IV (RD=-0.48; 95% CI: -0.67, -0.30; p<0.00001). For the outcomes, prednisone dosage ≤10 mg/day for ≥8 weeks was 48% (RD=0.48; 95% CI: 0.35 to 0.62; p<0.00001), and the eosinophil count <600/µL for ≥8 weeks was 51% (RD=0.51; 95% CI: 0.38 to 0.63; p<0.00001), both showed a reduction with MEP 300 mg/IV and 750 mg/IV. No statistically significant differences in treatment-related adverse effects outcomes were observed for either dosage (RD=0.09; 95% CI: -0.05 to 0.24; p=0.20; RD=0.09; 95% CI: -0.11 to 0.29; p=0.39). Despite the positive effects observed for the studied outcomes, the exact significance remains unclear.

Mepolizumab in hypereosinophilic syndrome: a systematic review and meta-analysis

We aimed to evaluate the efficacy and safety of mepolizumab (MEP) in the management of hypereosinophilic syndrome (HES). A systematic search was performed, and articles published until March 2021 were analyzed. The primary efficacy results evaluated were hospitalization rate related to HES, morbidity (new or worsening), relapses/failure, treatment-related adverse effects, prednisone dosage ≤10 mg/day for ≥8 weeks, and eosinophil count <600/μL for ≥8 weeks. A meta-analysis was conducted, when appropriate. Three randomized controlled trials (RCTs), with a total of 255 patients, were included. The studies contemplated the use of MEP 300 mg/SC or 750 mg/IV. According to the evaluation of the proposed outcomes, when relapse rates/therapeutic failures were assessed, there was a 26% reduction with MEP 300 mg/SC (RD=-0.26; 95% CI: -0.44 to -0.08; p=0.04) and 48% reduction with MEP 750 mg/IV (RD=-0.48; 95% CI: -0.67, -0.30; p<0.00001). For the outcomes, prednisone dosage ≤10 mg/day for ≥8 weeks was 48% (RD=0.48; 95% CI: 0.35 to 0.62; p<0.00001), and the eosinophil count <600/μL for ≥8 weeks was 51% (RD=0.51; 95% CI: 0.38 to 0.63; p<0.00001), both showed a reduction with MEP 300 mg/IV and 750 mg/IV. No statistically significant differences in treatment-related adverse effects outcomes were observed for either dosage (RD=0.09; 95% CI: -0.05 to 0.24; p=0.20; RD=0.09; 95% CI: -0.11 to 0.29; p=0.39). Despite the positive effects observed for the studied outcomes, the exact significance remains unclear.

Síndrome hipereosinofílico con eritrodermia / Hypereosinophilic syndrome with erythrodermia

Varón de 77 años de edad, con antecedentes de insuficiencia cardiaca y fibrilación auricular recibiendo warfarina, hipotiroidismo, diabetes mellitus e hiperuricemia, con historia de un año de lesiones dérmicas eritematosas y descamativas en el tronco, pruriginosas y con moderada eosinofilia recurrente. Después de descartarse causas alérgicas, parasitarias, autoinmunes y neoplásicas, se hizo el diagnóstico de síndrome hipereosinofílico idiopático. Recibió tratamiento con prednisona e hidroxiurea, consiguiéndose una remisión completa de la eosinofilia y mejoría progresiva de las lesiones dérmicas. (AU)

A 77-year-old male, with a history of heart failure and atrial fibrillation receiving warfarin, hypothyroidism, diabetes mellitus and hyperuricaemia, with a one-year history of erythematous and desquamative skin lesions in the trunk, pruritic and moderate recurrent eosinophilia. After allergic, parasitic, autoimmune and neoplastic causes were ruled out, the diagnosis of idiopathic hypereosinophilic syndrome was done. He was treated with prednisone and hydroxyurea, resulting in complete remission of eosinophilia and progressive improvement of dermal lesions. (AU)

Eosinophilic esophagitis: Current concepts in diagnosis and treatment.

Eosinophilic esophagitis is an immune-allergic pathology of multifactorial etiology (genetic and environmental) that affects both pediatric and adult patients. Its symptoms, which include heartburn, regurgitation, and esophageal stenosis (with dysphagia being more frequent in eosinophilic esophagitis in young adults and children), are similar to those of gastroesophageal reflux disease, causing delays in diagnosis and treatment. Although endoscopic findings such as furrows, esophageal mucosa trachealization, and whitish exudates may suggest its presence, this diagnosis should be confirmed histologically based on the presence of more than 15 eosinophils per high-power field and the exclusion of other causes of eosinophilia (parasitic infections, hypereosinophilic syndrome, inflammatory bowel disease, among others) for which treatment could be initiated. Currently, the 3 "D"s ("Drugs, Diet, and Dilation") are considered the fundamental components of treatment. The first 2 components, which involve the use of proton pump inhibitors, corticosteroids, immunosuppressants and empirical diets or guided food elimination based on allergy tests, are more useful in the initial phases, whereas endoscopic dilation is reserved for esophageal strictures. Herein, the most important aspects of eosinophilic esophagitis pathophysiology will be reviewed, in addition to evidence for the various treatments.

[Hypoxic hepatitis in a patient with endomyocardial fibrosis]. / Hepatitis hipóxica en paciente con endomiocardiofibrosis.

Endomyocardial fibrosis is a restrictive cardiomyopathy with high morbidity and mortality rates, prevalent in the sub-Saharan Africa region but infrequent in our population. It has a close relation with blood hypereosinophilia. Hypoxic hepatitis is frequently observed in intensive care units and its diagnosis is clinical. It shows a typical enzyme pattern with high mortality too. There are multiple mechanisms responsible for this condition, such as ischemia, passive congestion and dysoxia. We described the case of a 35 year-old cocaine addict woman diagnosed with endomyocardial fibrosis and hypereosinophilic syndrome who developed cardiogenic shock with hypoxic hepatitis. The patient evolved favorably with the appropriate treatment.

La endomiocardiofibrosis es una causa de miocardiopatía restrictiva frecuente en la región de áfrica subsahariana, aunque poco frecuente en nuestra población. Posee estrecha relación con la presencia de hipereosinofilia en sangre y tiene alta morbimortalidad. La hepatitis hipóxica es una afección clínica con un patrón enzimático característico, muy prevalente en unidades de cuidados intensivos y elevada mortalidad. Se reconocen múltiples mecanismos fisiopatológicos, como la isquemia, la congestión venosa y la alteración en la utilización de oxígeno del hepatocito. Describimos el caso de u na paciente de 35 años, consumidora de cocaína, con diagnóstico de endomiocardiofibrosis secundario a síndrome hipereosinofílico idiopático que presentó shock cardiogénico y hepatitis hipóxica asociada. Evolucionó favorablemente con el tratamiento de sostén adecuado.

Hepatitis hipóxica en paciente con endomiocardiofibrosis / Hypoxic hepatitis in a patient with endomyocardial fibrosis

La endomiocardiofibrosis es una causa de miocardiopatía restrictiva frecuente en la región de África subsahariana, aunque poco frecuente en nuestra población. Posee estrecha relación con la presencia de hipereosinofilia en sangre y tiene alta morbimortalidad. La hepatitis hipóxica es una afección clínica con un patrón enzimático característico, muy prevalente en unidades de cuidados intensivos y elevada mortalidad. Se reconocen múltiples mecanismos fisiopatológicos, como la isquemia, la congestión venosa y la alteración en la utilización de oxígeno del hepatocito. Describimos el caso de u na paciente de 35 años, consumidora de cocaína, con diagnóstico de endomiocardiofibrosis secundario a síndrome hipereosinofílico idiopático que presentó shock cardiogénico y hepatitis hipóxica asociada. Evolucionó favorablemente con el tratamiento de sostén adecuado.

Endomyocardial fibrosis is a restrictive cardiomyopathy with high morbidity and mortality rates, prevalent in the sub-Saharan Africa region but infrequent in our population. It has a close relation with blood hypereosinophilia. Hypoxic hepatitis is frequently observed in intensive care units and its diagnosis is clinical. It shows a typical enzyme pattern with high mortality too. There are multiple mechanisms responsible for this condition, such as ischemia, passive congestion and dysoxia. We described the case of a 35 year-old cocaine addict woman diagnosed with endomyocardial fibrosis and hypereosinophilic syndrome who developed cardiogenic shock with hypoxic hepatitis. The patient evolved favorably with the appropriate treatment.

Hypereosinophilic syndrome in an infant / Síndrome hipereosinofílico en un lactante

Abstract Background The hypereosinophilic syndrome (HES) is defined by an eosinophilic count > 1500 cell/mm3 and organ damage or dysfunction that can be easily mistaken for atopic dermatitis or pulmonary pathologies. Timely diagnosis and treatment can improve the prognosis and avoid heart and renal complications or lung fibrosis. Case report The case of an infant is reported with a 24-h evolution of cough and fever, personal history of atopic dermatitis, and a generalized dermatosis 2 months earlier. In the initial approach, respiratory disease was considered. However, blood count reported hypereosinophilia, which led to further studies and the diagnosis of the HES. Conclusions Although a rare pathology, it is important to consider the HES in children with common symptoms, and unusual evolution or poor treatment response and persistent hypereosinophilia.

Resumen Introducción El síndrome hipereosinofílico se define por la cuenta de eosinófilos > 1,500 células/mm3 con daño orgánico o disfunción, sin ninguna causa subyacente. Puede ser fácilmente confundido con una dermatitis atópica o con patologías pulmonares. El diagnóstico temprano y el tratamiento adecuado pueden mejorar el pronóstico y evitar complicaciones cardíacas y renales o el desarrollo de fibrosis pulmonar. Caso clínico Se reporta el caso de un lactante con tos y fiebre de 24 horas de evolución y una historia personal de dermatitis atópica, además de dermatosis generalizada dos meses antes. Inicialmente, se consideró como una enfermedad respiratoria; sin embargo, la cuenta de células sanguíneas reportó hipereosinofilia, lo cual condujo a estudios confirmatorios y al diagnóstico de síndrome hipereosinofílico. Conclusiones A pesar de ser una enfermedad rara, es de suma importancia considerar el síndrome hipereosinofílico en el diagnóstico diferencial en niños con una evolución atípica o con pobre respuesta al tratamiento, además de hipereosinofilia persistente.

Hypereosinophilic syndrome in an infant.

Background: The hypereosinophilic syndrome (HES) is defined by an eosinophilic count > 1500 cell/mm3 and organ damage or dysfunction that can be easily mistaken for atopic dermatitis or pulmonary pathologies. Timely diagnosis and treatment can improve the prognosis and avoid heart and renal complications or lung fibrosis. Case report: The case of an infant is reported with a 24-h evolution of cough and fever, personal history of atopic dermatitis, and a generalized dermatosis 2 months earlier. In the initial approach, respiratory disease was considered. However, blood count reported hypereosinophilia, which led to further studies and the diagnosis of the HES. Conclusions: Although a rare pathology, it is important to consider the HES in children with common symptoms, and unusual evolution or poor treatment response and persistent hypereosinophilia.

Introducción: El síndrome hipereosinofílico se define por la cuenta de eosinófilos > 1,500 células/mm3 con daño orgánico o disfunción, sin ninguna causa subyacente. Puede ser fácilmente confundido con una dermatitis atópica o con patologías pulmonares. El diagnóstico temprano y el tratamiento adecuado pueden mejorar el pronóstico y evitar complicaciones cardíacas y renales o el desarrollo de fibrosis pulmonar. Caso clínico: Se reporta el caso de un lactante con tos y fiebre de 24 horas de evolución y una historia personal de dermatitis atópica, además de dermatosis generalizada dos meses antes. Inicialmente, se consideró como una enfermedad respiratoria; sin embargo, la cuenta de células sanguíneas reportó hipereosinofilia, lo cual condujo a estudios confirmatorios y al diagnóstico de síndrome hipereosinofílico. Conclusiones: A pesar de ser una enfermedad rara, es de suma importancia considerar el síndrome hipereosinofílico en el diagnóstico diferencial en niños con una evolución atípica o con pobre respuesta al tratamiento, además de hipereosinofilia persistente.

Clinical, laboratory and therapeutic aspects of canine idiopathic hypereosinophilic syndrome: case report

The idiopathic hypereosinophilic syndrome (IHES) is a rare disease, characterized by variable eosinophilia and its massive infiltration into various organs. This study aimed to report clinical-laboratory findings and therapy in a canine with IHES. A one-year, 10-month-old male Rottweiler dog had a history of emesis, weight loss, hyporexia, and persistent eosinophilia on prior hematological exams. Vaccinations and worming were up to date. A complete blood count, serum biochemistry, bone marrow cytology, serology for leishmaniasis, chromatographic immunoassay for the detection of Dirofilaria immitis antigen, and abdominal ultrasound were requested. The tests for infectious diseases were negative. Blood biochemistry revealed no significant changes. An intense eosinophilia was observed in the hematology. A large number of cell precursors of the eosinophilic lineage were detected in the bone marrow cytology. Abdominal ultrasound showed thickening of intestinal loops. Considering the clinical and laboratory findings, the diagnosis of IHES was defined. Prednisolone treatment was instituted. The recurrence of peripheral eosinophilia occurred on the 35th day after therapy initiation. At that time, we opted to suspend the use of prednisolone and indicate the administration of deflazacort. With follow-up, therapeutic success with deflazacort was demonstrated, promoting the complete regression of clinical and ultrasound signs. The last glucocorticoid was maintained, but with a gradual dose reduction. The recognition of clinical and laboratory manifestations related to canine IHES is essential to establish an adequate diagnosis and therapy. Deflazacort emerges as a promising drug for controlling this disease.(AU)

Clinical, laboratory and therapeutic aspects of canine idiopathic hypereosinophilic syndrome: case report

The idiopathic hypereosinophilic syndrome (IHES) is a rare disease, characterized by variable eosinophilia and its massive infiltration into various organs. This study aimed to report clinical-laboratory findings and therapy in a canine with IHES. A one-year, 10-month-old male Rottweiler dog had a history of emesis, weight loss, hyporexia, and persistent eosinophilia on prior hematological exams. Vaccinations and worming were up to date. A complete blood count, serum biochemistry, bone marrow cytology, serology for leishmaniasis, chromatographic immunoassay for the detection of Dirofilaria immitis antigen, and abdominal ultrasound were requested. The tests for infectious diseases were negative. Blood biochemistry revealed no significant changes. An intense eosinophilia was observed in the hematology. A large number of cell precursors of the eosinophilic lineage were detected in the bone marrow cytology. Abdominal ultrasound showed thickening of intestinal loops. Considering the clinical and laboratory findings, the diagnosis of IHES was defined. Prednisolone treatment was instituted. The recurrence of peripheral eosinophilia occurred on the 35th day after therapy initiation. At that time, we opted to suspend the use of prednisolone and indicate the administration of deflazacort. With follow-up, therapeutic success with deflazacort was demonstrated, promoting the complete regression of clinical and ultrasound signs. The last glucocorticoid was maintained, but with a gradual dose reduction. The recognition of clinical and laboratory manifestations related to canine IHES is essential to establish an adequate diagnosis and therapy. Deflazacort emerges as a promising drug for controlling this disease.

Case for diagnosis. Erythroderma as manifestation of hypereosinophilic syndrome.

Hypereosinophilic syndrome is defined as persistent eosinophilia (>1500/µL for more than six months) associated with organ involvement, excluding secondary causes. It is a rare, potentially lethal disease that should be considered in cutaneous conditions associated with hypereosinophilia. We report a case of erythroderma as a manifestation of hypereosinophilic syndrome. A 36-year-old male with no comorbidities presented progressive erythroderma, pruritus, peripheral neuropathy, and eosinophilia in the previous seven months. No mutations were found in FIP1L1/PDGFRA. Patient experienced rapid remission in response to oral prednisone and hydroxyurea. Cutaneous manifestations may be the only evidence of hypereosinophilic syndrome. Genotyping excludes myeloproliferative disease, thereby orienting treatment and prognosis.

Case for diagnosis. Erythroderma as manifestation of hypereosinophilic syndrome

Abstract: Hypereosinophilic syndrome is defined as persistent eosinophilia (>1500/µL for more than six months) associated with organ involvement, excluding secondary causes. It is a rare, potentially lethal disease that should be considered in cutaneous conditions associated with hypereosinophilia. We report a case of erythroderma as a manifestation of hypereosinophilic syndrome. A 36-year-old male with no comorbidities presented progressive erythroderma, pruritus, peripheral neuropathy, and eosinophilia in the previous seven months. No mutations were found in FIP1L1/PDGFRA. Patient experienced rapid remission in response to oral prednisone and hydroxyurea. Cutaneous manifestations may be the only evidence of hypereosinophilic syndrome. Genotyping excludes myeloproliferative disease, thereby orienting treatment and prognosis.

Endomiocardite de Loeffler ­ manifestação cardíaca da síndrome hipereosinofílica: relato de caso / Loeffler's endomyocarditis ­ cardiac manifestation of hypereosinophilic syndrome: case report'

Introdução: A síndrome hipereosinofílica é caracterizada por uma produção aumentada e contínua de eosinófilos e pode levar a lesões teciduais em múltiplos órgãos, como consequência da infiltração eosinofílica. Os pacientes apresentam eosinofilia absoluta no sangue periférico (> 1.500 eosinófilos/mm3) sem uma causa primária de eosinofilia. A manifestação cardíaca desta síndrome geralmente se apresenta como endomiocardite de Loeffler, que constitui uma miocardiopatia restritiva primária resultante da infiltração de eosinófilos no tecido cardíaco. Descrição do caso: Relatamos o caso raro de uma paciente de 64 anos com eosinofilia a esclarecer e comprometimento cardíaco, que teve o diagnóstico estabelecido a partir de exames de imagem. Comentários: Enfatizamos os aspectos clínicos e evolutivos, ressaltando as dificuldades diagnósticas e a importância da investigação de eosinofilias persistentes sem causa aparente, uma vez que o diagnóstico e tratamento precoce podem proporcionar melhores taxas de sobrevida e prognóstico nestes pacientes.

Introduction: The hypereosinophilic syndrome is characterized by an increased, continuous production of eosinophils, and it may lead to tissue damage in multiple organs as a consequence of eosinophilic infiltration. Patients with this syndrome present absolute eosinophil count > 1,500 eosinophils/mm3 in the peripheral blood without a primary cause for eosinophilia. The cardiac manifestation of this syndrome usually presents as Loeffler's endomyocarditis, a primary restrictive cardiomyopathy resulting from the infiltration of eosinophils into cardiac tissue. Case description: We report the rare case of a 64-year-old woman with eosinophilia and cardiac involvement, who had the diagnosis established based on imaging tests. Comments: We emphasize the clinical and evolutionary aspects of the condition, highlighting the diagnostic difficulties and the importance of investigating persistent eosinophilia without an apparent cause, as early diagnosis and treatment can provide better survival rates and improved prognosis in these patients.

Usefulness of three-dimensional echocardiography in the assessment of valvular involvement in Loeffler endocarditis.

Loeffler endocarditis is a complication of hypereosinophilic syndrome resulting from eosinophilic infiltration of heart tissue. We report a case of Loeffler endocarditis in which three-dimensional transthoracic and transesophageal echocardiography provided additional information to what was found by two-dimensional transthoracic echocardiography alone. Our case illustrates the usefulness of combined two- and three-dimensional echocardiography in the assessment of Loeffler endocarditis. In addition, a summary of the features of hypereosinophilic syndrome and Loeffler endocarditis is provided in tabular form.