Clinical characteristics, ultrasonic diagnosis, treatment and outcomes of eosinophilic fasciitis: a retrospective single-center analysis of 45 cases / 南方医科大学学报

OBJECTIVE@#To evaluate the clinical features, laboratory and imaging results, treatment and outcomes of eosinophilic fasciitis (EF) and assess the value of ultrasound in the diagnosis of EF.@*METHODS@#We retrospectively analyzed the clinical data of 45 patients with EF treated in our center from January 1, 2006 to February 28, 2022. The consistency between the diagnoses of EF based on ultrasound and MRI findings was assessed.@*RESULTS@#In the 45 EF patients (male/female ratio 3.5:1), the age of onset ranged from 16 to 64 years with a mean disease course of 22.6 months. The average time from symptom onset to diagnosis was 16 months. The most common possible trigger of the disease was vigorous exercise (10/45), causing symmetrical lesions in the limbs, most commonly in the forearms (86.7%) and lower legs (80%). Clinical features of EF included subcutaneous swelling and induration (95.6%), arthralgia and arthritis (55.6%), groove sign (42.2%), hand joint contractures (42.2%), skin pigmentation (37.8%), and peau d'orange appearance (13.3%). Eosinophilia was found in 31 patients (68.9%). Hypergammaglobulinemia was seen in 23/44 (52.3%) and positive antinuclear antibodies in 9 (20%) of the patients. Twentyone of the patients were treated with high-dose methylprednisolone (≥200 mg daily for 3 to 5 consecutive days), and compared with the patients who did not receive this treatment, these patients more frequently experienced relapse before admission, had more extensive involvement, and had a higher rate of hypergammaglobulinemia without fever, but these differences were not statistically significant. Of the 31 patients (68.9%) with follow-up data (for a median of 3.2 years [range 0.2-15.9]), complete remission was achieved in 12 (38.7%) patients, and the accumulative complete remission rate was 44.1% at 5.5 years. No specific baseline characteristics or immunosuppressants were found to correlate with the treatment response. A total of 26 patients underwent both ultrasound and MRI examination, and the Kappa value of the diagnostic results between ultrasound and MRI was 0.91.@*CONCLUSION@#EF is characterized by symmetrical subcutaneous swelling and induration in the limbs, accompanied by eosinophilia and hypergammaglobulinemia. Glucocorticoid is effective for treating EF. Ultrasound examination can identify thickening of subcutaneous fascia for an early diagnosis of EF.

Eosinophilic gastroenteritis: Pathogenesis, diagnosis, and treatment / 中华医学杂志(英文版)

Eosinophilic gastroenteritis (EGE) is a gastrointestinal disorder of unclear etiology that is characterized by eosinophilic infiltration of the stomach and small intestine, and consists of mucosal, muscular, and serosal subtypes. Eosinophilic infiltration of the gastrointestinal tract is a fundamental histopathological characteristic of EGE and is driven by several T-helper type 2 (Th2)-dependent cytokines and induced by food allergy. Due to the lack of a diagnostic gold standard, EGE has a high rate of delayed diagnosis or misdiagnosis. However, several new diagnostic strategies have been developed, such as novel genetic biomarkers and imaging tests. Although dietary therapy and corticosteroids remain the common choices for EGE treatment, recent decades have seen the emergence of novel treatment alternatives, such as biologics that target particular molecules involved in the pathogenic process. Preliminary investigations and clinical trials have demonstrated the efficacy of biologics and provided additional insights for the era of refractory or corticosteroid-dependent EGE biologics.

Risk Factors and Prognosis of Cardiovascular Damage in Hypereosinophilia / 中国实验血液学杂志

OBJECTIVE@#To investigate the risk factors and prognosis of cardiovascular damage in hypereosinophilia (HE).@*METHODS@#The clinical data of 62 patients with HE in Gansu Provincial Hospital from January 2015 to December 2020 were retrospectively analyzed, including clinical characteristics and laboratory indicators, and the influencing factors of survival and prognosis were also analyzed.@*RESULTS@#In this study, there were 34 males and 28 females, with a median age of 53.5 (20-79) years, 35 patients without cardiovascular damage, 27 patients with cardiovascular damage, including 22 patients with abnormal electrocardiogram (ECG) (81.5%), 18 patients with abnormal echocardiography (ECHO) (66.7%), 9 patients with single ECG abnormality, 5 patients with single ECHO abnormality, and other 13 patients with multiple abnormalities. In cardiovascular damage group, peripheral white blood cell count, absolute value of eosinophils, troponin T (TNT), N-terminal pro-B-type natriuretic peptide (NT-proBNP), interleukin (IL)-4 and IL-5 levels at initial diagnosis were significantly higher than those in the non-cardiovascular damage group (P <0.01), while hemoglobin, IL-2 and interferon-γ levels were significantly lower (P <0.01). There were no significant differences in age, sex, course of disease, etiological classification, platelet count, serum creatine kinase, serum creatine kinase isoenzyme and lactate dehydrogenase between the two groups (P >0.05). The 5-year overal survival rate of patients with cardiovascular damage was 88.9%, and that of patients without cardiovascular damage was 100%, the difference was statistically significant (P =0.012). The 5-year event-free survival (EFS) rate of patients with cardiovascular damage was 59.3%, and the median time was 37 (21-52) months, while that of patients without cardiovascular damage was 80%, and the median time was 63 (51-74) months (P =0.002). Age (>60 years old), course of disease (>24 months), NT-proBNP (>3 000 pg/ml), TNT (>100 ng/L), elevated IL-4 and IL-5 were associated with EFS shortening in patients with cardiovascular damage, which were independent risk factors for EFS.@*CONCLUSION@#The EFS rate in HE patients without cardiovascular damage is significantly higher than patients with cardiovascular damage. Age, course of disease, NT-proBNP, TNT, IL-4 and IL-5 are independent risk factors affecting EFS of patients with cardiovascular damage.

Granuloma ulcerativo traumático con eosinoflia estromal: lesión simuladora de difícil diagnóstico. Presentación de caso / Traumatic ulcerative granuloma with stromal eosinophilia: Simulator lesión of difcult diagnosis. Presentation of a case

Introducción: el granuloma ulcerativo traumático con eosinofilia estromal es una afección benigna, crónica y autolimitante, que por su evolución clínica puede estar sujeta a confusión diagnóstica. Por ello, el caso que aquí se comparte expone particularidades de esta afección y su respuesta al tratamiento para permitir un mejor conocimiento de esta lesión. Se describen las características clínicas e histopatológicas y su evolución ante la terapéutica empleada. Se presenta una paciente femenina de seis años, con antecedentes de salud y de dientes neonatales, que hace tres meses manifiesta dos úlceras en mucosa sublingual que no cicatrizan ni mejoran al tratamiento anterior. Se indicaron estudios hematológicos, se realizó biopsia incisional de la lesión con su estudio histopatológico e inmunohistoquímico. Se obtuvo eosinofilia estromal y ausencia de linfocitos anómalos CD30. El tratamiento incluyó aplicación de corticosteroides tópicos e intralesionales, experimentando remisión de la lesión. Se mantiene la paciente bajo seguimiento clínico, sin recidiva de lesión. Conclusiones: el granuloma ulcerativo traumático con eosinofilia estromal es una lesión autolimitante que puede ser confundida con otras lesiones ulcerativas de la cavidad bucal, por lo que su estudio histopatológico resulta imprescindible para su diagnóstico. Asimismo, su análisis inmunohistoquímico es indispensable para precisar su naturaleza y probable evolución. El adecuado y exhaustivo proceso diagnóstico constituye una herramienta vital para lograr su identificación.

Background: Traumatic ulcerative granuloma with stromal eosinophilia is a benign, chronicle and self-healing lesion, which can be misdiagnosed for its clinical evolution, for this reason, this case report is carried out, showing particularities of this disease and its response in front to the treatment, giving a better identifcation of the lesion, were described the clinical and histopathological fndings of a case. A feminine patient, six years old, with neonatal health and dental history. She has presented during three months two ulcerative lesions in sublingual mucosa, which do not improve with the previous treatment. Hematological studies and biopsy were carried out, the incisional biopsy was analyzed with immunohistochemical test, the results were stromal eosinophilia and absence of anomalous lymphocytes CD30. She was treated with topical and intralesional corticosteroids, experiencing remission of the lesion. The patient had a long clinical follow up without recidive. Traumatic ulcerative granuloma with stromal eosinophilia is a self-healed lesion that needs a histopathological and inmunohistochemical analysis for an adequate diagnosis. The correct diagnostic sequence is a vital tool to achieve its identification.

Colitis eosinofílica asociada a enfermedad inflamatoria intestinal / Eosinophilic colitis associated with inflammatory bowel disease

INTRODUCCIÓN: La colitis eosinofílica y la colitis de la enfermedad inflamatoria intestinal, son dos entidades que pueden compartir similares características clínicas, endoscópicas y terapéuticas pero diferentes criterios diagnósticos. OBJETIVOS: Describir el caso clínico de un niño preescolar con antecedente de alergia alimentaria, de hospitalizaciones y uso de antibióticos por varias ocasiones, que evoluciona con diarrea crónica intermitente. CASO CLÍNICO: Se trata de un paciente masculino, de 3 años 5 meses, con antecedente de alergia alimentaria con cuadro crónico de dolor abdominal, diarrea y retraso en el crecimiento. Se realiza abordaje de diarrea crónica. RESULTADOS: Con hallazgos clínicos de enfermedad inflamatoria intestinal y descripción histopatológica de colitis eosinofílica, se considera la asociación entre estas dos patologías sin dejar la posibilidad de que esta última se trate de una fase inicial de enfermedad inflamatoria intestinal. CONCLUSIONES: El tratamiento de pacientes con colitis eosinofílica complicada es similar a la enfermedad inflamatoria intestinal, se requiere seguimiento clínico, endoscópico e histopatológico de pacientes con colitis eosinofílica a largo plazo.

INTRODUCTION: Eosinophilic colitis and inflammatory bowel disease colitis are two entities that may share similar clinical, endoscopic and therapeutic features but different diagnostic criteria. OBJECTIVES: To describe the clinical case of a preschool child with a history of food allergy, hospitalizations and use of antibiotics for several occasions, who evolves with chronic intermittent diarrhea. CLINICAL CASE: This is a male patient, 3 years 5 months old, with a history of food allergy with chronic abdominal pain, diarrhea and growth retardation. Chronic diarrhea was approached. RESULTS: With clinical findings of inflammatory bowel disease and histopathological description of eosinophilic colitis, the association between these two pathologies is considered without leaving the possibility that the latter is an initial phase of inflammatory bowel disease. CONCLUSIONS: The treatment of patients with complicated eosinophilic colitis is similar to inflammatory bowel disease, clinical, endoscopic and histopathological follow-up of patients with eosinophilic colitis is required in the long term.

Síndrome DRESS inducido por ácido valproico en una niña que respondió a la ciclosporina con análisis de HLA / Valproic acid-induced DRESS in a child responding to cyclosporine with HLA analysis

El síndrome de erupción medicamentosa con eosinofilia y síntomas sistémicos (drug reaction with eosinophilia and systemic symptoms, DRESS), también conocido como síndrome de hipersensibilidad inducida por medicamentos, es una reacción rara potencialmente mortal que causa una erupción grave y que puede provocar insuficiencia multiorgánica. Como con otras erupciones medicamentosas graves, los linfocitos T específicos para un medicamento tienen una función crucial en el síndrome DRESS. El modelo de hapteno/pro-hapteno, el modelo de interacción farmacológica y el modelo alterado de repertorio de péptidos son tres modelos diferentes desarrollados para describir la relación/interacción entre un medicamento o sus metabolitos y el sistema inmunitario. Analizamos nuestra experiencia con el tratamiento con ciclosporina en un caso de síndrome DRESS resistente a esteroides causado por ácido valproico en una niña y sus resultados clínicos, de laboratorio y de antígeno leucocitario humano (HLA).

Drug reaction with eosinophilia and systemic symptoms (DRESS), also known as drug-induced hypersensitivity syndrome, is a potentially life-threatening rare reaction that causes a severe rash and can lead to multiorgan failure. As in other severe drug eruptions, drug-specific T lymphocytes play a crucial role in DRESS. The hapten/pro-hapten model, pharmacological interaction model, and altered peptide repertoire model are three different models developed to describe the relationship/interaction between a medication or its metabolites and the immune system. We discuss our experience with cyclosporine treatment in a steroid-resistant DRESS syndrome caused by valproic acid in a girl, as well as her clinical, laboratory, and human leukocyte antigens (HLA) study results

Reacción a fármacos con eosinofilia y síntomas sistémicos (DRESS) / Drug reaction with eosinophilia and systemic symptoms (DRESS)

La reacción a fármacos con eosinofilia y síntomas sistémicos (del acrónimo en inglés DRESS: drug reaction with eosinophilia and systemic symptoms) o síndrome de hipersensibilidad inducida por fármacos (del acrónimo en inglés DIHS: drug induced hypersensitivity syndrome), es una reacción adversa a fármacos (RAF) grave e infrecuente. Los mecanismos involucrados en su fisiopatogenia incluyen diversas alteraciones de las enzimas metabolizadoras de fármacos, con la consecuente acumulación de metabolitos reactivos, la reactivación secuencial de virus de la familia del herpes, la predisposición genética asociada a ciertos alelos de antígenos leucocitarios humanos (HLA) y una respuesta de hipersensibilidad retardada de tipo IV. En la actualidad, se han ido incorporando nuevos fármacos responsables de este cuadro como medicamentos biológicos, inmunosupresores y quimioterápicos. La presentación clínica del DRESS es variable. Tiene una morbimortalidad alta y supone costos elevados en la atención médica. Su tratamiento consiste, en primer lugar, en la suspensión de los fármacos causales o sospechosos de desencadenar el síndrome. Luego, según la gravedad del cuadro, se pueden indicar corticosteroides sistémicos o inmunoglobulina (IGIV) combinada con corticosteroides, plasmaféresis, ciclosporina, micofenolato de mofetilo y rituximab. El objetivo de este trabajo fue realizar una revisión sobre el DRESS y destacar los aspectos nuevos y relevantes de los últimos 5 años.

Drug reaction with eosinophilia and systemic symptoms (DRESS) or drug-induced hypersensitivity syndrome (DIHS), is a serious and rare adverse drug reaction. Among the mechanisms involved in its pathophysiology, there are various alterations in drugmetabolizing enzymes with the consequent accumulation of reactive metabolites, sequential reactivation of viruses of the herpes family, genetic predis-position associated with certain HLA, and a type IV hypersensitivity response. Currently, new drugs responsible for this pathology have been incorporated, such as biologicals, immunosuppressants and chemotherapy. The clinical presentation of DRESS is variable. It has a high morbidity and mortality and involves high costs in medical care. Its treatment consists, in the first place, in the suspension of the causal or suspected drugs. Then, depending on severity, systemic corticosteroids or IVIG combined with corticosteroids, plasmapheresis, cyclosporine, mycophenolate mofetil, and rituximab may be indicated.The objective of this work was review DRESS and highlight the new and relevant aspects of the last 5 years.

Hereditary angioedema and Allergic bronchopulmonary aspergillosis: an unexpected association / Angioedema hereditário e Aspergilose broncopulmonar alérgica: uma associação inesperada

Hereditary angioedema (HAE) is a rare autosomal dominant disorder, Allergic bronchopulmonary aspergillosis (ABPA) is a lung disease involving hypersensitivity to the fungi Aspergillus fumigatus which occur in susceptible patient with asthma or cystic fibrosis, also considered a rare disease. We report a case of HAE and ABPA in a single patient. HAE diagnosis was confirmed: C4 = 3 mg/dL, C1INH < 2.8 mg/dL - nephelometry. Former lung function showed elevation RV and RV/FVC, suggesting small airways lung disease. Positive skin prick test to Aspergillus fumigatus (03 mm); total serum IgE level 3,100 IU/mL (nephelometry - BNII Siemens), eosinophilia 11% (528/mm3) and specific A. fumigatus IgG antibodies 6,8 mgA/L (FEIA - fluorenzymeimmunoassay - ThermoFisher) and Chest CT showed mucoid impaction of the bronchi, consistent to current ABPA. Controlling ABPA could prevent and reduce angioedema attacks, and lung structural damage. Early diagnosis and treatment of both diseases should be emphasized to reduce mortality and morbidity

Angioedema hereditário (AEH) é uma doença autossômica dominante; aspergilose broncopulmonar alérgica (ABPA) é uma doença de hipersensibilidade pulmonar relacionada ao esporo de Aspergillus fumigatus, mais suscetível em pacientes com asma e fibrose cística, ambas são consideradas doenças raras. Apresentamos um caso de AEH e ABPA em um paciente. O diagnóstico de AEH foi confirmado com exames laboratoriais: C4 = 3 mg/dL, C1INH < 2,8 mg/dL - nefelometria. Prova de função pulmonar evidenciou aumento de VR e VR/CVF, sugerindo doenças de pequenas vias aéreas. Teste de puntura positivo para A. fumigatus (03 mm); IgE total = 3.100 IU/mL (nefelometria - BNII Siemens), eosinofilia 11% (528/mm3) e IgG específica para A. fumigatus 6,8 mgA/L (FEIA - ThermoFisher), TC de tórax evidenciou impactação mucoide, consistente com ABPA. Controlar ABPA pode prevenir e reduzir as crises de angioedema e os danos ao tecido pulmonar. O diagnóstico precoce de ambas as doenças deve ser enfatizado para reduzir a morbimortalidade.

Síndrome DRESS secundária ao uso de alopurinol: um relato de caso / DRESS syndrome secondary to the use of allopurinol: a case report

A Síndrome de DRESS (do inglês, Drug Rash with Eosinophilia and Systemic Symptoms) é uma patologia rara que consiste em uma severa reação medicamentosa mediada por células T. O presente relato de caso retrata uma paciente do sexo feminino, 59 anos, que apresentou icterícia, febre não termometrada, acolia, colúria, mialgia, placas hipercrômicas e lesões pruriginosas. Referiu uso recente de alopurinol, paracetamol e nimesulida, apresentando melhora importante e espontânea após a suspensão das medicações. A extensão do tempo de exposição ao medicamento agressor ocasiona um maior período de internação e risco de mortalidade. Além disso, os dados restritos sobre a Síndrome de DRESS impõe desafios ao seu diagnóstico. Sendo assim, este estudo busca destacar a importância do diagnóstico clínico precoce, a suspensão do medicamento agressor e a instituição da terapêutica adequada para um prognóstico favorável

The Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) Syndrome is a rare pathology that consists of a severe drug reaction mediated by T cells. The present case report depicts a female patient, 59 years old, who presented jaundice, non thermometered fever, acholia, choluria, myalgia, hyperchromic plaques and pruritic lesions. She mentioned recent use of allopurinol, paracetamol and nimesulide, showing significant and spontaneous improvement after discontinuation of medications. The extension of time of exposure to the offending drug causes a longer period of hospitalization and risk of mortality. In addition, the restricted data on DRESS Syndrome poses challenges to its diagnosis. Therefore, this study seeks to highlight the importance of early clinical diagnosis, suspension of the offending drug and the institution of appropriate therapy for a favorable prognosis

Fibrosis endomiocárdica: una revisión actualizada a propósito de unos casos en un programa de falla cardiaca / Endomyocardial fibrosis: an updated review, about some cases in a heart failure program

Resumen Introducción La fibrosis endomiocárdica es una enfermedad cardiovascular olvidada, principal causa de cardiomiopatía restrictiva. Se estima que en el mundo hay 10-12 millones de personas con la enfermedad. La mayoría se restringen a zonas de África, Asia y, en Latinoamérica, en Brasil y Colombia. La etiología y la fisiopatología son poco comprendidas. Para su diagnóstico se deben tener en cuenta los datos del paciente, el cuadro clínico y los hallazgos ecocardiográficos. Presentamos los casos confirmados en un centro de referencia cardiovascular. Descripción de los casos: Se han documentado tres casos, de los cuales dos son mujeres. Todos debutaron con un cuadro insidioso de disnea con esfuerzo. Los hallazgos ecocardiográficos fueron disfunción diastólica con patrón restrictivo, insuficiencia moderada de válvulas auriculoventriculares e hipertensión pulmonar; en dos casos se encontraron imágenes anormales endocárdicas en el ventrículo izquierdo y se realizó biopsia endomiocárdica para confirmar el diagnóstico. Ninguno mejoró la clase funcional ni la frecuencia de hospitalizaciones. Conclusiones La fibrosis endomiocárdica debe considerarse en todo paciente con falla cardiaca con un patrón restrictivo asociado y con poca respuesta al manejo farmacológico convencional. Los pacientes presentados tenían hallazgos típicos de la enfermedad, con la particularidad de ser de edades mayores.

Abstract Introduction Endomyocardial fibrosis is one of the neglected cardiovascular disease, the main cause of restrictive cardiomyopathy. There are an estimated 10-12 million people with the disease worldwide. Most are restricted to areas of Africa, Asia and in Latin America in Brazil and Colombia. The etiology and pathophysiology are poorly understood. For its diagnosis, the patient's data, the clinical picture and the echocardiographic findings must be taken into account. We present the confirmed cases in a cardiovascular referral center. Description of the cases Three cases have been documented of which two are women. All debuted with insidious dyspnea on exertion. The echocardiographic findings were diastolic dysfunction with a restrictive pattern, moderate atrioventricular valve insufficiency, and pulmonary hypertension. In two cases, endocardial abnormal images were found in the left ventricle and an endomyocardial biopsy was performed to confirm the diagnosis. None improved functional class or frequency of hospitalizations. Conclusions Endomyocardial fibrosis should be considered in a patient with heart failure with an associated restrictive pattern, and with little response to conventional pharmacological management. The patients presented had typical findings of the disease with the particularity of being older.

Presentación atípica de toxocariasis infantil: a propósito de un caso / Atypical presentation of infant toxocariasis: about a case

Resumen Se presenta el caso de una niña de 8 años, procedente de la Amazonia peruana, con una presentación atípica de una toxocariasis. El cuadro clínico, de un mes de evolución, fue caracterizado por dolor abdominal crónico, palidez y geofagia, asociado a una masa abdominal y linfoadenopatías generalizadas. No hubo sintomatología respiratoria, dérmica, ocular o neurológica. En el hemograma se encontró una anemia y eosinofilia periférica acentuada y una eosinofilia moderada en el aspirado medular. El diagnóstico fUe confirmado por serología por el método de enzimoinmunoanálisis (ELISA), que mostró la presencia de anticuerpos anti-Toxocara de tipo IgG e IgM. Se administró tratamiento con albendazol 400 mg al día, durante cinco días, con una evolución favorable.

Abstract We present the case of an 8-year-old girl with an atypical presentation of toxocariasis, from the Peruvian Amazon. A month ago, the clinical presentation was characterized by the presence of abdominal pain, paleness and geophagia, associated with an abdominal mass and generalized lymphadenopathy. There were no respiratory, dermal, ocular or neurological symptoms. Marked peripheral eosinophilia was found in the blood count, and moderate eosinophilia in the spinal cord aspírate. The diagnosis was confirmed by serology with the enzyme-linked immunosorbent assay (ELISA), which showed the presence of IgG and IgM anti- Toxocara antibodies. Treatment with albendazole 400 mg once daily was administered for five days with favorable evolution.

Drug Reaction with eosinophilia and systemic symptoms with alopecia universalis and vitiligo / Reação a drogas com eosinofilia e sintomas sistêmicos associada a alopecia universal e vitiligo

ABSTRACT: Drug reactions with eosinophilia and systemic symptoms (DRESS) are rare and potentially fatal adverse hypersen-sitivity reaction to some drugs, especially anticonvulsants.The syndrome affects not only the skin but also other organs, especially the liver. The incidence can vary from 1 to 5 cases per 10.000 patients exposed to anticonvul-sants. The recognition of the syndrome is of fundamental importance since the mortality rate is between 10 and 40%. Once the diagnosis is established, the triggering drug must be identified and discontinued. Corticosteroids are usually associated with therapy. Autoimmune sequelae have been reported, including vitiligo and rarely alopecia. Alopecia universalis is a variant of alopecia areata, characterized by hair loss throughout the body. We report a case of DRESS, associated with two autoimmune dermatological diseases: alopecia universalis and vitiligo. (AU)

RESUMO: A reação a drogas com eosinofilia e sintomas sistêmicos (DRESS) é uma rara e potencialmente fatal reação adversa de hipersensibilidade, decorrente de alguns medicamentos, principalmente os anticonvulsivantes. A síndrome não afeta apenas a pele, mas também outros órgãos, principalmente o fígado. A incidência pode variar de 1 a 5 casos por 10.000 pacientes expostos aos anticonvulsivantes. O reconhecimento da síndrome é de fundamental importân-cia devido a taxa de mortalidade entre 10-40%. Uma vez estabelecido o diagnóstico, deve-se identificar o medica-mento desencadeante e suspendê-lo. O corticosteróide geralmente é associado na terapia. Sequelas autoimunes foram relatadas, incluindo vitiligo e raramente alopecia. A alopecia universal é uma variante da alopecia areata, caracterizada pela perda de pelos em todo o corpo. Relatamos um caso de DRESS, associado a duas doenças au-toimunes dermatológicas: alopecia universal e vitiligo. (AU)

Mepolizumab as a steroid-sparing treatment option in a patient with eosinophilic granulomatosis with polyangiitis / Mepolizumabe como terapêutica poupadora de esteroides num paciente com granulomatose eosinofílica com poliangiite

Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem disorder characterized by asthma, peripheral blood eosinophilia, and signs of vasculitis. Glucocorticoids are considered the cornerstone of treatment, but most patients remain steroid-dependent and carry a significant burden of adverse effects. We report a case of a patient with steroid-dependent EGPA successfully treated with mepolizumab. A 36-year-old man presented with persistent rhinitis, dyspnea, wheezing, and dry cough poorly controlled with inhaled therapy. Eosinophilia in peripheral blood and bronchoalveolar lavage fluid was seen. Histological findings from nasal mucosa revealed eosinophilic microabscesses and vasculitis without granulomas compatible with EGPA diagnosis. After daily oral prednisolone (PSL) was started, symptoms and eosinophilia improved, but adverse effects emerged. Attempts at tapering off PSL resulted in worsening of symptoms. He started mepolizumab 300 mg monthly, with clinical improvement and sustained disease remission, which allowed reducing the need for PSL. We present a very disabling steroiddependent EGPA. Mepolizumab was able to taper off PSL while maintaining symptomatic control.

Granulomatose eosinofílica com poliangiite (EGPA) é uma doença multissistêmica caracterizada por asma, eosinofilia no sangue periférico e sinais de vasculite. Os corticoides são considerados a base do tratamento, no entanto, a maioria dos pacientes permanece dependente deste tratamento com os seus efeitos adversos associados. Relatamos o caso de um paciente com granulomatose eosinofílica dependente de esteroides com poliangiite (EGPA) tratado com sucesso com mepolizumabe. Um homem de 36 anos apresentou rinite persistente, dispneia, sibilos e tosse seca mal controlada com terapia inalada. Observou-se eosinofilia no sangue periférico e no lavado broncoalveolar. Os achados histológicos da mucosa nasal revelaram microabscessos eosinofílicos e vasculite sem granulomas compatíveis com o diagnóstico de EGPA. Após o início da prednisolona oral diária (PSL), os sintomas e a eosinofilia melhoraram, mas surgiram efeitos adversos. As tentativas de redução gradual da PSL resultaram no agravamento dos sintomas. Iniciou mepolizumabe 300 mg mensalmente, com melhora clínica e remissão sustentada da doença, o que permitiu reduzir a necessidade de PSL. Apresentamos um EGPA dependente de esteroides muito incapacitante. O mepolizumab foi capaz de diminuir o PSL mantendo o controle sintomático sustentado.

Síndrome de Reacción a drogas con Eosinofilia y Síntomas Sistémicos (DRESS) asociados a Nefritis Túbulo Intersticial Aguda en un adolescente / DRESS síndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) associated with Tubulo Interstitial Nephritis in a adolescent

Resumen El síndrome de DRESS (Reacción a drogas con eosinofilia y síntomas sistémicos) es una patología poco frecuente en Pediatría, descrita por primera vez en 1996, por Bocquet. Puede presentarse en un tiempo variable luego de exposición a algunos medicamentos, se caracteriza por fiebre, compromiso cutáneo y de órganos internos. En este caso, se presenta a un paciente de 13 años, con antecedente de uso de Trimetroprim sulfa desde hace 2 meses, con cuadro de 3 días consistente en fiebre y rash cutáneo, sin compromiso de mucosas, con respuesta no favorable al manejo con esteroide, requiriendo Inmunoglobulina IV. Semanas después del inicio de los síntomas y evolución estable presenta insuficiencia renal aguda que requirió terapia de reemplazo renal. Se descartaron otras patologías subyacentes de índole autoinmune. Hubo recuperación de azoados y normalización de los demás paraclínicos el día 40 de la enfermedad. El paciente continúa asintomático, 4 meses después, con tratamiento con esteroide oral, en descenso lento y gradual. Se debe considerar la evaluación permanente de las pruebas de función renal en los pacientes que presenten Síndrome de DRESS, por su asociación con Nefritis intersticial aguda y complicaciones relacionadas.

Abstract DRESS syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) is a rare pathology in Pediatrics, first described in 1996 by Bocquet. It can appear in a variable period of time after exposure to some medications, it is characterized by fever, skin involvement and internal organs. A 13-year-old patient is presented, with a history of use of Trimethoprim sulfa for two months, with a disease of three days of evolution, consisting of fever and skin rash, without mucosal involvement, with an unfavorable response to steroid management, requiring Intravenous inmunoglobulin. Weeks after the onset of symptoms and stable evolution, he presented acute renal failure that required renal replacement therapy. Other underlying autoimmune pathologies were ruled out. There was recovery of renal function test and normalization of the other paraclinical on day 40 of the disease. Patient remains asymptomatic four months later, with oral steroid treatment, in slow and gradual decline. Permanent evaluation of renal function tests should be considered in patients with DRESS syndrome, due to its association with acute tubulointerstitial nephritis and related complications.

Síndrome de DRESS en una paciente con fibrosis quística: reporte de un caso pediátrico / DRESS syndrome in a patient with cystic fibrosis: a pediatric case report

El síndrome de sensibilidad a fármacos con eosinofilia y síntomas sistémicos o síndrome de DRESS según sus siglas en inglés (drug reaction with eosinophilia and systemic symptoms) se encuentra entre las reacciones medicamentosas cutáneas graves. Este consiste en una tríada clínica que incluye fiebre, exantema y compromiso sistémico, acompañado de eosinofilia y/o linfocitos atípicos.Se presenta el caso de una paciente de sexo femenino con fibrosis quística, de 18 meses de edad, quien desarrolló esta patología durante un tratamiento con trimetoprima-sulfametoxazol para erradicar Staphylococcus aureus meticilino resistente en esputo. Los pacientes con fibrosis quística reciben múltiples esquemas antibióticos según bacteriología en secreciones respiratorias para evitar el deterioro de la función pulmonar y colonización por gérmenes resistentes. Es menester conocer y sospechar este síndrome, debido al riesgo incrementado de hipersensibilidad a drogas en fibrosis quística, pronóstico ominoso y su elevada morbimortalidad

Drug reaction with eosinophilia and systemic symptoms or DRESS syndrome is among severe cutaneous drug reactions. This constitutes a clinical triad that includes fever, skin rash and systemic compromise, accompanied by eosinophilia and/or atypical lymphocytes.We present the case of an 18-month-old female patient with cystic fibrosis, who develops this pathology during a trimethoprim-sulfamethoxazole cycle as an eradicating treatment of methicillin-resistant Staphylococcus aureus in bronchial secretions. Cystic fibrosis patients receive multiple antibiotic regimens according to bacteriology in sputum, to avoid impairment in their lung function and colonization by resistant germs. Due to the increased risk of drug hypersensitivity in cystic fibrosis, an ominous prognosis and high morbidity and mortality, knowledge and a high index of suspicion of this syndrome are necessary

Síndrome de reacción a drogas con eosinofilia y síntomas sistémicos asociado a herpesvirus humano 6. Caso pediátrico tratado con ciclosporina y corticoides / Drug reaction with eosinophilia and systemic symptoms syndrome associated with human herpesvirus 6. A pediatric case treated with cyclosporine and corticosteroids

La reacción a drogas con eosinofilia y síntomas sistémicos es una reacción adversa cutánea rara, potencialmente grave. Puede presentar fiebre, erupción cutánea polimorfa, edema facial y/o linfoadenopatías. La reactivación del virus herpes humano tipo 6 se asocia a un curso más grave y/o prolongado.Un lactante de 22 meses en tratamiento con fenobarbital presentó lesiones eritematopapulares, fiebre, leucocitosis, proteína C reactiva elevada y alteración de pruebas hepáticas. Se realizó biopsia de piel compatible con reacción adversa a drogas. Se trató con corticoides sistémicos e inmunoglobulina intravenosa sin respuesta. La reacción en cadena de la polimerasa para virus herpes humano tipo 6 resultó positiva. Se inició ciclosporina más prednisona, con buena respuesta. Existe poca evidencia del uso de ciclosporina en adultos, cuando los corticoides sistémicos son inefectivos. Este es el primer reporte pediátrico Podría ser una alternativa efectiva o un complemento de los corticosteroides sistémicos cuando no responde a tratamientos convencionales.

Drug reaction with eosinophilia and systemic symptoms is a rare and potentially serious skin adverse reaction, with fever, polymorphous skin rash, facial edema, and/or lymphadenopathy. Reactivation of human herpes virus type 6 has been associated with a more severe and/or prolonged course. A 22-month-old infant under phenobarbital treatment developed erythematous-papular lesions, fever, leukocytosis, elevated C-reactive protein, and abnormal liver tests. The skin biopsy was compatible with an adverse drug reaction. Treatment with systemic corticosteroids and intravenous immunoglobulin had no response. Polymerase chain reaction for human herpesvirus type 6 was positive, and cyclosporine plus prednisone was started with a good response. There is little evidence for the use of cyclosporine in adults when systemic corticosteroids are ineffective. This is the first report of pediatric drug reaction with eosinophilia and systemic symptoms treated with cyclosporine, which could be an effective alternative or an adjunct to systemic corticosteroid therapy unresponsive to conventional treatments.

Traumatic ulcerative granuloma with stromal eosinophilia, an unusual clinical manifestation in the oral mucosa: case report / Granuloma ulcerativo traumático con eosinofilia estromal, una manifestación clínica inusual en la mucosa oral: reporte de un caso

Introduction: Traumatic ulcerative granuloma with stromal eosinophilia is an uncommon condition of the oral mucosa with a chronic course, usually affecting the tongue. Case Report: Clinically it presents as a chronic ulcer, with raised and indurated borders, rarely presented as a tumor. Histologically it shows a diffuse mixed inflammatory infiltrate, rich in eosinophils. The etiology of this lesion is still unclear; however, chronic irritation from traumatic agents is considered a major initiating factor. In some cases, the presence of CD30+ mononuclear cells within the lesions suggest the possibility of a CD30+ lymphoproliferative disorder. This article presents a case of a traumatic ulcerative granuloma with stromal eosinophilia manifested in a 56-year-old female with a solitary ulcerated tumor inside the right cheek. Conclusion: It was diagnosed based on clinical data and histopathological features. In a brief literature review, the entity has been characterized, analyzing its etiology and nature.

Introducción: El granuloma ulcerativo traumático con eosinofilia estromal es una afección infrecuente de la mucosa oral de curso crónico, que suele afectar a la lengua. Case Report: Clínicamente se presenta como una úlcera crónica, con bordes elevados e indurados, rara vez se presenta como un tumor. Histológicamente muestra un infiltrado inflamatorio mixto difuso, rico en eosinófilos. La etiología de esta lesión aún no está clara; sin embargo, la irritación crónica por agentes traumáticos se considera un factor de iniciación importante. En algunos casos, la presencia de células mononucleares CD30 + dentro de las lesiones sugiere la posibilidad de un trastorno linfoproliferativo CD30+. En este artículo se presenta el caso de un granuloma ulcerativo traumático con eosinofilia estromal que se manifiesta en una mujer de 56 años con un tumor ulcerado solitario en el interior de la mejilla derecha. Conclusión: Se diagnosticó con base en datos clínicos y características histopatológicas. En una breve revisión de la literatura se ha caracterizado la entidad, analizando su etiología y naturaleza.

Síndrome DRESS asociado a fenitoína: informe de caso / DRESS syndrome associated with phenytoin: case report

El síndrome DRESS es una reacción adversa dermatológica que puede presentarse debido a diversos medicamentos, y constituye uno de los diagnósticos más importantes por encima del síndrome de Stevens-Johnson. Se trata de un caso relacionado con una reacción adversa de muy baja frecuencia, que está documentada en la literatura científica, a varios medicamentos, entre ellos la fenitoína. Por lo mencionado, la publicación de estos casos resulta escasa y limitada. Las principales preocupaciones del paciente relacionadas con su cuadro clínico radicaban en el gran compromiso cutáneo que lo llevó a hospitalización, dolor e incomodidad, por el cual recurrió al manejo tópico generalizado con vaselina. Los hallazgos clínicos relevantes fueron: eosinofilia severa, ulceraciones cutáneas, hepatitis química y fiebre. Con los hallazgos del cuadro clínico y la evaluación de la escala RegiSCAR se hace el diagnóstico de síndrome DRESS inducido por fenitoína. Se suspende la fenitoína, se inicia levetiracetam y se administran corticosteroides y acetaminofén con evolución favorable. (AU)

DRESS syndrome is a dermatological adverse reaction can occur due to various medications, being one of the most important diagnoses above Steven-Johnson syndrome. This is a case related to a very low frequency adverse reaction that is documented in the scientific literature to several medicines among those, the phenytoin. Therefore, the publication of these cases is scarce and limited. The main concerns of the patients related to their clinical picture were due to the great cutaneous compromise that lead to hospitalization, pain and discomfort for which they resorted to generalized topical management with vaseline (petrolatum). Relevant clinical findings were severe eosinophilia, skin ulcerations, chemical hepatitis and fever. With clinical picture findings and evaluation of the RegiSCAR scale, the diagnosis of Phenytoin-induced DRESS syndrome is made. Phenytoin is discontinued, levetiracetam is started and corticosteroids and acetaminophen are administrated with favorable evolution. (AU)

Episodic angioedema with eosinophilia (Gleich's syndrome) associated with urticarial vasculitis: a coincidence or a novel clinical entity?

Episodic angioedema with eosinophilia (EAE) is a rare condition characterized by recurrent attacks of angioedema and urticaria accompanied by a marked elevation of peripheral eosinophil count. We report the case of a young female patient diagnosed with EAE associated with urticarial vasculitis. A 40-year-old female patient was admitted to our institution due to recurrent episodes of cheek and eyelid angioedema in the previous year. Episodes of facial angioedema lasted for two months with spontaneous remission afterwards. In addition, she presented pruritic and painful skin eruptions of erythematous circles, which persisted for longer than 24 h, that were palpable, somewhat purplish, and more pronounced on the face, arms, and trunk. Laboratory investigation showed a sustained elevation of white cell counts with marked eosinophilia. Serum IgM, IgE, and IgA were normal; IgG was slightly elevated. C1-esterase inhibitor and tryptase test were normal. Reverse transcriptase-polymerase chain reaction was performed for detection of FIP1L1-PDGFRA and BCR-ABL rearrangements. None of these alterations were found. Skin biopsies were suggestive of urticarial vasculitis. The patient was submitted to esophagogastroduodenoscopy, which showed mild chronic gastritis, with no eosinophilic infiltration. Cardiac dimensions and function were normal. Abdominal ultrasound and total body CT-scan failed to show lymphadenopathy, organomegaly, and tumors. We report the first case of association between episodic angioedema with eosinophilia and urticarial vasculitis. It is possible that both conditions share a physiopathological mechanism, suggesting that it is not just a chance association.