Pustulosis subcórnea: a propósito de un caso / Subcorneal pustulosis: a case report

Resumen La dermatosis pustulosa subcórnea (DPS), oenfermedad de Sneddon-Wilkinson, es una dermatosis poco frecuente, benigna, de curso crónico y recidivante. En esta oportunidad presentaremos el caso de una paciente femenina de 58 años condiagnóstico de dermatosis pustulosa subcórnea, que fue tratada con dapsona 100mg por día, con resolución de lesiones. Realizamos unarevisión bibliográfica de esta patología describiendo sus características clínicas e histológicas, sus múltiples diagnósticos diferenciales y las posibles terapéuticas.

Abstract Subcorneal pustular dermatosis, or Sneddon-Wilkinson disease, is a rare, benign, chronic and recurrent dermatosis. We reportthe case of a 58-year-old female patient with subcorneal pustular dermatosis, who was treated with dapsone 100mg per day, with goodtherapeutic response. We carried out a bibliographic review of this pathology describing its clinical and histological characteristics, its multiple differential diagnoses and possible therapeutic options.

Skin Rash as the First Manifestation of Pediatric Chronic Neutrophilic Leukemia / 罕见病研究

A 6-year-old girl presented with recurrent skin rash at the initial stage, recent joint pain, and neutrophilia was found during a routine blood test. After a multidisciplinary case discussion, she was diagnosed with chronic neutrophil leukemia, and the symptoms were relieved after hydroxyurea and luxolitinib treatment. She received the allogeneic hematopoietic stem cell transplantation subsequently. At present, she is in stable condition and under follow-up. Chronic neutrophil leukemia is a rare disease, which rarely occurs in children. It is more difficult to diagnose in patients with skin rash as the first manifestation. The diagnosis and treatment of this case reflects the important role of multidisciplinary cooperation in the diagnosis and treatment of difficult and rare diseases.

Study the mechanism of Dingchuan decoction in neutrophilic asthma mice on serum metabolomics / 中国医师杂志

Objective:Based on the method of metabonomics, the intervention effect of Dingchuan decoction on neutrophilic asthma and its possible mechanism were analyzed from the changes of endogenous metabolites.Methods:Forty C57BL/6 mice were randomly divided into five groups: normal group (group A), neutrophilic asthma model group (group B), Dingchuan decoction low dose treatment group (group C), Dingchuan decoction medium dose treatment group (group D), and Dingchuan decoction high dose treatment group (group E), with 8 mice in each group.B/C/D/E group used ovalbumin (OVA) and complete Freund′s adjuvant (CFA) to induce sensitized mice to establish neutrophilic asthma model, and C/D/E group used Dingchuan decoction with different concentrations of crude drugs for intervention treatment.Buxco small animal lung function tester was used to evaluate the airway reactivity of mice; The total number of white blood cells in bronchoalveolar lavage fluid (BALF) was counted by counting plate, and the number was classified by cell smear staining; The pathological changes of lung tissue were observed by hematoxylin and eosin (HE) staining.The serum metabolites of mice in each group were detected by high performance liquid chromatography coupled with four pole time of flight mass spectrometry (UPLC-Q-TOF-MS/MS). The commonly used principal component analysis (PCA), partial least squares discriminant analysis (PLS-DA), orthogonal partial least squares discriminant analysis (OPLS-DA) models were used for statistical analysis of the metabolite profiles in serum. The intervention effect of Dingchuan decoction and its possible mechanism were reflected from the changes of endogenous metabolites.Results:(1) General behavior observation: except mice in group A, mice in other groups showed asthma symptoms of different degrees during the challenge period. The symptoms of mice in each treatment group (group C, D, E) of Dingchuan decoction were less than those in group B. (2) Airway reactivity: the airway reactivity of mice in group B to methacholine (MCh) increased with the inhalation concentration, and the airway resistance at each concentration of MCh was significantly higher than that in group A (all P<0.01); the airway reactivity in group C, D and E was lower than that in group B (all P<0.01); the airway reactivity in group D and E was lower than that in group C (all P<0.05). There was no significant difference in airway reactivity between group D and E ( P>0.05). (3) Airway inflammatory cell infiltration: the total number of white blood cells (WBC) and percentage of neutrophil in BALF of group B were significantly higher than those of group A (all P<0.01). The total WBC and percentage of neutrophil in group C, D and E were lower than those in group B (all P<0.01). The total number of WBC and percentage of neutrophil in group D and E were lower than those in group C (all P<0.01). There was no significant difference between group D and group E (all P>0.05). (4) Pathological changes of lung tissues: no pathological changes were observed in the lung tissues of group A mice. In group B, typical pathological changes such as bronchial lumen stenosis, intraluminal mucosal folds hyperplasia, epithelial cell exfoliation, swelling, mucous embolus, alveolar and lung tissue structure destruction, massive inflammatory cell infiltration around bronchus and blood vessels were observed, among which neutrophil and lymphocyte infiltration were the most obvious. The damage of lung tissue structure, bronchial mucosa edema and inflammatory cell infiltration in Dingchuan decoction treatment groups were significantly improved compared with group B, and the pathological changes of lung tissue in group D were relatively light. (5) Metabolomics analysis: PCA, PLS-DA and OPLS-DA analysis of serum metabolites in each group showed that serum metabolites in group A and group B were significantly different. The metabolic pathway analysis showed that Dingchuan decoction with different crude drug concentrations could improve the metabolic disorders caused by asthma in different degrees. Conclusions:Dingchuan decoction can effectively reduce airway inflammation and airway hyperresponsiveness in mice with neutrophil asthma, and effectively regulate metabolic abnormalities caused by neutrophil asthma.

Mastitis granulomatosa idiopática. Características clínicas, imagenológicas, patológicas y su correlación con la respuesta al tratamiento - Hospital Dr. Cosme Argerich / Idiopathic granulomatous mastitis: clinical, imagenological, pathological characteristics and their correlation with treatment response

Introducción: La mastitis granulomatosa idiopática (MGI) es una patología inflamatoria benigna que se presenta típicamente como tumoración dolorosa. Recientemente, un subconjunto con patrón histológico neutrofilico quístico (MGNQ) fue asociado al corynebacterium. Objetivo: Revisión bibliográfica y evaluación de nuestra casuística. Material y método: Se seleccionaron 24 mujeres con MGI diagnosticada entre 2000-2019. Variables analizadas: demográficas, antecedentes, clínica, imágenes, cultivos, patología, tratamientos y evolución. Resultados: Edad media: 40.7 años. 50% tuvo ≥4 gestas y 91.6% lactó. Presentación clínica: Tumor abscedado palpable 58.33%, Palpable asintomático 25%, Palpable doloroso 12.5%. Lesión no palpable 4.17%. Tamaño promedio de lesión: 3.5 cm. Imágenes BIRADS 4/5: 87.5% Cultivo de corynebacterium 25%. Patología: Polimorfonucleares intraductales (PMNID) 62.5%, Vacuolas quísticas 50% (2 casos con baci- los). Tratamiento: Antibióticoterapia 79.1%, Inmunosupresión 79.1%, Drenaje de abscesos 45.8%. Tumorectomía 41.6%. Tiempo de resolución: 5.5 meses Tiempo libre de enfermedad: 33.6 meses. Recurrencias: 31.%. Conclusiones: Debe sospecharse en toda mujer joven con tumor mamario asociado a abscesos, fistulas y/o úlceras. El diagnóstico de MGNQ y PM- NID tienen asociación estadísticamente significativa con recurrencia. Es esencial el abordaje multidisciplinario y el tratamiento multimodal

Introduction: Idiopathic granulomatous mastitis (IGM) is a benign inflammatory disease that typically presents as a painful mass. Recently, a subset with cystic neutrophilic histological pattern (CNGM) was related to corynebacterium. Objetive: Bibliographic review and evaluation of our casuistry. Material and method: During 2000-2019, 24 women diagnosed with IGM were selected. Variables analyzed: Demographic, background, clinical, images, cultures, pathology, treatment and evolution. Results: Average age: 40.7 years. 50% had ≥ 4 gestations and 91.6% lactated. Clinical presentation: Palpable abscessed tumor 58.33%, Palpable asymptomatic 25%, Palpable painful 12.5%. Non palpable lesion 4.17%. Average lesion size: 3.5 cm. BIRADS images 4/5: 87.5% Corynebacterium culture 25%. Findings: Intraductal polymorphonuclear cells (IDPMNC) (62.5% ), Cystic vacuoles (50%) (With bacilli in 2 cases). Treatment: Antibiotic therapy 79.1%, Immunosuppression 79.1%, Drainage of abscesses 45.8%. Lumpectomy 41.6%. Resolution time: 5.5 months Diseasefree time: 33.6 months. Recurrences: 31.8%. Conclusions: It should be suspected in any young woman with a breast tumor associated with abscesses, fistulas and/or ulcers. The diagnosis of CNGM and IDPMNC has statistically significant association with recurrence. A multidisciplinary approach and multimodal treatment is essential

Síndrome de SWEET. A propósito de 1 caso / SWEET syndrome. About 1 case

El Síndrome de Sweet llamado también dermatosis neutrofílica febril aguda es una enfermedad rara, de naturaleza inflamatoria, caracterizada por fiebre de inicio agudo, neutrofilia, lesiones cutáneas eritematosas y dolorosas, infiltrado típico de neutrófilos en la dermis superior y rápida mejoría con corticoesteroides sistémicos. Presenta formas típicas y atípicas, las primeras cumplen con todos los criterios de diagnóstico y las causas pueden ser neoplásica, infecciosa, fármacos, embarazo y a veces idiopática. Se presenta un caso de Síndrome de Sweet típico en una mujer, probablemente desencadenado por un cuadro infeccioso de vías aéreas superiores con confirmación histológica y buena respuesta a la corticoterapia, con remisión total y sin recidiva a la fecha

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a rare, inflammatory disease characterized by acute-onset fever, neutrophilia, erythematous and painful skin lesions, a typical neutrophil infiltrate in the upper dermis, and rapid improvement with systemic corticosteroids. It presents typical and atypical forms, the former meet all diagnostic criteria and the causes can be neoplastic, infectious, drugs, pregnancy, and sometimes idiopathic. We present a case of typical Sweet syndrome in a woman, probably triggered by an infectious picture of the upper airways with histological confirmation and good response to corticosteroid therapy, with total remission and no recurrence to date

Neutrophilic dermatosis of the dorsal hands related to cocaine abuse

Abstract Neutrophilic dermatoses encompass a wide spectrum of diseases characterized by a dense infiltration mainly composed of neutrophils. Neutrophilic dermatosis of the dorsal hands is currently considered a localized variant of Sweet syndrome. Cocaine abuse has been related to a wide range of mucocutaneous manifestations, including neutrophilic dermatoses such as pyoderma gangrenosum. The authors of this study present a patient with neutrophilic dermatosis of the dorsal hands, in which cocaine abuse was identified as a probable trigger.

Índice neutrófilo linfocitario en la predicción del infarto del miocardio tipo 4a / Lymphocyte neutrophilic index in the prediction of type 4 myocardial infarction

Introducción: El infarto del miocardio tipo 4a es una complicación del intervencionismo coronario percutáneo, que se asocia a un proceso inflamatorio. El índice neutrófilo linfocitario, como marcador de inflamación, pudiera incluirse en su estratificación del riesgo. Objetivos: Evaluar la sensibilidad, especificidad, los valores predictivos y la variación del índice neutrófilo linfocitario en la predicción del infarto del miocardio tipo 4a. Métodos: Investigación de cohorte prospectiva, en 184 pacientes a los que se les realizó intervencionismo coronario percutáneo. Resultados: Para un valor mayor e igual a 2,74, el índice mostró una sensibilidad de 72,0 por ciento, una especificidad de 74,8 por ciento un valor predictivo negativo de 94,4 por ciento en la predicción de infarto tipo 4a. La variación del índice fue predictor independiente de la complicación p < 0,001. Conclusiones: El índice neutrófilo linfocitario tiene alta sensibilidad, especificidad y valor predictivo negativo en la predicción del infarto tipo 4a. Su elevación a las seis horas del proceder es un predictor independiente para dicha complicación(AU)

Introduction: Type 4a myocardial infarction is a complication of percutaneous coronary intervention, which is associated with an inflammatory process. The lymphocyte neutrophilic index, as a marker of inflammation, could be included in your risk stratification. Objectives: To assess the sensitivity, specificity, predictive values and the variation of the lymphocyte neutrophilic index in the prediction of type 4a myocardial infarction. Methods: A prospective cohort investigation was carried in 184 patients who underwent percutaneous coronary intervention. Results: For a value higher than and equal to 2.74, the index showed 72.0 percent, sensitivity, 74.8 percent specificity and 94.4 percent negative predictive value in the prediction of type 4a infarction. The variation of the index was an independent predictor of the complication p < 0.001. Conclusions: The lymphocyte neutrophil index has high sensitivity, specificity and negative predictive value in the prediction of type 4a infarction. Its elevation six hours after the procedure is an independent predictor for this complication(AU)

Pioderma gangrenoso ampollar como forma de presentación de leucemia mieloide agudo: informe de un caso / Acute myeloid leukemia presented in the form of bullous pyoderma gangrenosum: a case report

El pioderma gangrenoso ampollar es una variedad infrecuente de pioderma gangrenoso, que se asocia en el 50-70% de los casos con trastornos oncohematológicos. Se comunica el caso de una paciente de 59 años, que consultó por fiebre y ampollas purpúricas de rápida progresión, con compromiso cutáneo mucoso. Con sospecha de una enfermedad neutrofílica, ampollar, o infección por gérmenes oportunistas, se realizó biopsia de piel para estudio histopatológico, inmunofluorescencia directa y cultivo. Los cultivos y la inmunofluorescencia directa fueron negativos, y la anatomía patológica reveló un denso infiltrado inflamatorio con predominio neutrofílico en dermis. Ante el diagnóstico de pioderma gangrenoso ampollar, se realizó una punción-aspiración de médula ósea cuyo resultado fue compatible con leucemia mieloide aguda. Se instauró tratamiento con corticosteroides sistémicos, a pesar de lo cual la paciente evolucionó desfavorablemente y falleció a los 15 días de su ingreso hospitalario. Este caso ilustra la asociación de esta enfermedad cutánea con trastornos oncohematológicos y el mal pronóstico que esto implica a corto plazo. (AU)

Bullous pyoderma gangrenosum is an infrequent type of pyoderma gangrenosum, associated with onco hematological diseases in 50-70% of cases. We present the case of a 59-year-old patient with fever and mucocutaneous hemorrhagic bullous of rapid progression. A biopsy for histopathology, direct immunofluorescence (DIF) and skin culture was made, considering the possibility of neutrophilic dermatoses, bullous dermatosis or an opportunistic infection. The results of both the culture and the DIF were negative. The histopathological examination of the specimen revealed a dense dermal polymorphic infiltrate composed primarily of neutrophils. Considering bullous pyoderma gangrenosum as a potential diagnosis, a bone-marrow biopsy was performed. This study revealed an acute myeloid leukemia. Although systemic corticosteroid therapy was begun, the patient presented an unfavorable evolution that led to her death 15 days after her admission at the hospital. This case shows the association between bullous pyoderma gangrenosum and onco hematological diseases. In addition, it highlights the poor prognosis related to these diseases in the short term. (AU)

Mastitis Quística Granulomatosa Neutrofílica, Reporte De Caso / Granulomatous Neutrophilic Cystic Mastitis, Case Report

La mastitis granulomatosa neutrofílica quística (MGNQ) es una entidad recientemente caracterizada, con detalles histopatológicos específicos que la diferencian de otros tipos de mastitis crónicas idiopáticas. La presencia de bacilos grampositivos dentro de espacios de apariencia quística rodeados de neutrófilos, en un contexto de inflamación granulomatosa supurativa, definen esta entidad. La importancia de su reconocimiento en el reporte diagnóstico recae en su asociación a la infección por especies de corinebacterias, de esa manera se puede direccionar el tratamiento con antibióticos, más allá del tratamiento antiinflamatorio que suele administrarse en mastitis idiopáticas. Se describe el caso de una paciente de sexo femenino, de 35 años de edad, sin antecedentes relacionados, con dolor mamario intermitente, asociado a la presencia de múltiples nodulaciones palpables, duras, en ambas mamas, con resultados de múltiples biopsias particulares previas que describieron mastitis crónica granulomatosa con reacción de tipo tuberculoso, por lo cual recibió tratamiento antituberculoso. Pese a ello, las tumoraciones cedieron sólo parcialmente. Se revisó las láminas histológicas en la institución y se estableció el diagnóstico de MGNQ. Se consideró importante la presentación de este caso debido a su escaso reconocimiento entre patólogos, pese a presentar características ya definidas en estudios previos.

Cystic neutrophilic granulomatous mastitis (CNGM) is a recently characterized entity, with specific histopathological details that differentiate it from other types of chronic idiopathic mastitis. The presence of gram-positive bacilli within cystic-like spaces surrounded by neutrophils, in a context of suppurative granulomatous inflammation, define this entity. The importance of its recognition in the diagnostic report lies in its association with infection by corynebacterial species, so that treatment with antibiotics can be targeted, beyond the anti-inflammatory treatment that is usually administered to idiopathic mastitis. We describe the case of a 35-year-old female patient with no related history, with intermittent breast pain, associated with the presence of multiple hard, palpable nodules in both breasts, with results from multiple previous private biopsies that described chronic granulomatous mastitis with a tubercular reaction, for which she received treatment for tuberculosis. Despite this, the clinical response was partial. Histological slides were reviewed at the institution and the diagnosis of CNGM was then established. The presentation of this case was considered important due to its low recognition among pathologists, despite presenting characteristics already defined by previous studies.

Mastitis quística granulomatosa neutrofílica asociada a corinebacterias: Reporte de caso

La mastitis granulomatosa neutrofílica quística (MGNQ) es una entidad recientemente caracterizada, con detalles histopatológicos específicos que la diferencian de otros tipos de mastitis crónicas idiopáticas. La presencia de bacilos grampositivos dentro de espacios de apariencia quística rodeados de neutrófilos, en un contexto de inflamación granulomatosa supurativa, definen esta entidad. La importancia de su reconocimiento en el reporte diagnóstico recae en su asociación a la infección por especies de corinebacterias, de esa manera se puede direccionar el tratamiento con antibióticos, más allá del tratamiento antiinflamatorio que suele administrarse en mastitis idiopáticas. Se describe el caso de una paciente de sexo femenino, de 35 años de edad, sin antecedentes relacionados, con dolor mamario intermitente, asociado a la presencia de múltiples nodulaciones palpables, duras, en ambas mamas, con resultados de múltiples biopsias particulares previas que describieron mastitis crónica granulomatosa con reacción de tipo tuberculoso, por lo cual recibió tratamiento antituberculoso. Pese a ello, las tumoraciones cedieron sólo parcialmente. Se revisó las láminas histológicas en la institución y se estableció el diagnóstico de MGNQ. Se consideró importante la presentación de este caso debido a su escaso reconocimiento entre patólogos, pese a presentar características ya definidas en estudios previos.

Cystic neutrophilic granulomatous mastitis (CNGM) is a recently characterized entity, with specific histopathological details that differentiate it from other types of chronic idiopathic mastitis. The presence of gram-positive bacilli within cystic-like spaces surrounded by neutrophils, in a context of suppurative granulomatous inflammation, define this entity. The importance of its recognition in the diagnostic report lies in its association with infection by corynebacterial species, so that treatment with antibiotics can be targeted, beyond the anti-inflammatory treatment that is usually administered to idiopathic mastitis. We describe the case of a 35-year-old female patient with no related history, with intermittent breast pain, associated with the presence of multiple hard, palpable nodules in both breasts, with results from multiple previous private biopsies that described chronic granulomatous mastitis with a tubercular reaction, for which she received treatment for tuberculosis. Despite this, the clinical response was partial. Histological slides were reviewed at the institution and the diagnosis of CNGM was then established. The presentation of this case was considered important due to its low recognition among pathologists, despite presenting characteristics already defined by previous studies.

Bioinformatics analysis on differential expression genes of neutrophilic asthma and drug screening / 中国药理学通报

Aim To explore the differential expression genes (DEGs) and potential therapeutic drugs of neutrophilic asthma (NA) based on bioinformatics analysis and molecular docking. Methods The gene expression profiles of NA were obtained from GEO database, and the differential expression genes were screened. The protein-protein interactions (PPI) of DEGs were obtained from STRING database, and the hub genes were screened by Cytoscape according to the degree of DEGs. The GO and KEGG pathway analysis were performed by DAVID database. Finally, molecular docking technology was used to screen the potential therapeutic drugs for the treatment of NA. Results A total of 147 DEGs were obtained from NA patients compared with healthy people in GEO database. Ten hub genes were screened from PPI network, including CXCL8, FPR2, CXCL1, TNFRSF1B, CXCR1, etc. Go enrichment analysis showed that DEGs were mostly associated with inflammation, immune response and chemotaxis, etc. KEGG pathway analysis indicated that the DEGs were mainly involved in cytokine-cytokine receptor interaction and complement and coagulation signaling pathways. Molecular docking showed that paeoniflorigenone and triptolide had good binding activity with C8B and PLAU. Conclusion Complement and coagulation cascades may become a new therapeutic target of NA. The two screened compounds paeoniflorigenone and triptolide may be potential therapeutic drugs for the treatment of NA.

The role of neutrophils in asthma / 浙江大学学报·医学版

Bronchial asthma is a chronic respiratory disease，characterized by airway inflammation，airway hyperresponsiveness，reversible airway obstruction and airway remodeling，in which a variety of cells including airway inflammatory cells and structural cells are involved. Previous studies have shown that asthma is mainly driven by Th2 cytokines IL-4，IL-5，and IL-13，leading to airway eosinophil inflammation. With further research，however，it has been found that neutrophils are also closely related to asthma. Numbers of neutrophils are elevated in airway through increased chemotaxis and decreased apoptosis，which is earlier than eosinophils，leading to airway neutrophilic inflammation. Neutrophils can produce elastase，myeloperoxidase，neutrophil extra- cellular traps，chemokines and cytokines，participating in the occurrence and development of asthma. The antagonists against these molecules，such as anti-IL-8 receptor antibody，anti-IL-17 antibody，and DNase，have shown positive effects on neutrophilic asthma，but further studies are needed to support their clinical application. This article mainly reviews the role of neutrophils in asthma and related mechanisms.

lgA pemphigus, intraepidermal neutrophillic type in an 8-year old Filipino female: A case report

INTRODUCTION@#lgA pemphigus is a rare, chronic, relapsing, benign group of autoimmune intraepidermal blistering dermatosis with an unknown etiology. It is characterized by significantly pruritic, vesiculopustular lesions that occur mainly on the trunk and proximal extremities. Histopathologic and immunofluorescence studies show intraepidermal blisters and deposition of immunoglobulin A in the intercellular spaces of the epidermis, respectively.@*CASE REPORT@#To our knowledge, we present the first reported pediatric case of lgA pemphigus, intraepidermal neutrophilic type, in an 8-year old Filipino female with a 2-year history of generalized papules and flaccid pustules, some forming an annular pattern. Diagnosis was confirmed by histopathology and direct immunofluorescence. Enzyme-linked immunosorbent assay for Desmoglein 1 was negative. Complete clearance of lesions was achieved with dapsone, colchicine and prednisone.

Pyoderma Gangrenosum Arising De Novo Over an Unusual Site: A Case Report

Summary@#Pyoderma gangrenosum (PG) of the breast is a rare rapidly progressive neutrophilic dermatosis, which usually co-exists with severe underlying systemic conditions. A woman presented with a non-healing ulcer over her right breast with characteristic sparing of nipple-areola complex (Bork-Baykal phenomenon). It was diagnosed as pyoderma gangrenosum on the basis of clinico-pathological correlation and managed successfully with systemic corticosteroids and anti-inflammatory drugs along with wound care. The diagnosis and treatment of PG is challenging particularly at unusual sites given the paucity of robust clinical evidence and lack of consensus opinion regarding specific management guidelines. It is imperative that PG is considered as a clinical diagnosis in any patient with enlarging, sterile, necrotic lesions unresponsive to appropriate antibiotics. Early recognition of PG at rare locations can prevent devastating sequelae such as over-zealous surgical debridement and deep tissue infections associated with a chronic open wound leading to severe cosmetic morbidity.

Caracterización clínica e inmunológica del Asma Neutrofílica / Clinical and immunological characterization of Neutrophilic asthma

RESUMEN Introducción: El asma bronquial es una enfermedad mucho más compleja de lo que inicialmente se consideraba, es multifactorial y se manifiesta en diferentes fenotipos clínicos, entre ellos, el asma neutrofílica. Objetivo: Caracterizar clínica e inmunológicamente el asma neutrofílica. Material y Métodos: Se realizó una revisión bibliográfica actualizada. Se empleó el motor de búsqueda Google Académico y se consultaron artículos de libre acceso en las bases de datos Pubmed y Scielo y se revisaron 50 artículos. Desarrollo: Los neutrófilos están presentes en las vías respiratorias del paciente con asma, se activan y pueden liberar mediadores que promueven y prolongan los síntomas del asma, la presencia de neutrófilos puede ser mediada por interleucina 17(IL-17), la hipótesis de cambios en la microbiota, la obesidad y las infecciones, constituyen elementos primordiales en este tipo de enfermedad. Para aliviar los síntomas de estos pacientes, se necesita una terapia dirigida a disminuir la inflamación dominada por neutrófilos. Conclusiones: El asma neutrofílica es una enfermedad crónica fenotípicamente heterogénea de las vías respiratorias, que implica la participación de numerosas células inflamatorias y más de cien mediadores con múltiples efectos inflamatorios, como el broncoespasmo, la exudación plasmática, la hipersecreción de moco y la activación sensorial. Actualmente existen pocas drogas dirigidas contra la inflamación neutrofílica y la mayoría se encuentran en estudio con el objetivo de aliviar y mejorar las condiciones de vida de cada uno de los pacientes que sufren esta enfermedad(AU)

ABSTRACT Introduction: Bronchial asthma is a much more complex disease than initially thought; it is multifactorial and manifests itself in different clinical phenotypes; among them, we can mention neutrophilic asthma. Objective: To characterize neutrophilic asthma from a clinical and immunological point of view. Material and methods: An updated bibliographic review was made. Google Scholar search engine was used; free access articles were consulted in PubMed and SciELO databases and 50 of them were reviewed. Development: Neutrophils are present in the respiratory tract of patients suffering from asthma; once those neutrophils are activated, they can release mediators that promote and prolong the symptoms of the disease. The presence of neutrophils can be mediated by IL-17. The hypothesis on changes in the microbiota, obesity and infections are key points in this kind of disease. To alleviate the symptoms of these patients, targeted therapy for neutrophil-dominated inflammation is needed. Conclusions: Neutrophilic asthma is a phenotypically heterogeneous chronic disease of the respiratory tract which involves numerous inflammatory cells and more than a hundred mediators with multiple inflammatory effects such as bronchospasm, plasma exudation, mucus hypersecretion and sensory activation. Currently, there are few drugs targeting neutrophilic inflammation and most of them are under study with the aim of alleviating and improving the living conditions in patients suffering from this disease(AU)

Interstitial granulomatous dermatitis: a rare case report of dermatological manifestation of rheumatoid arthritis

Interstitial granulomatous dermatitis (IGD), a rare dermatological disorder was first described by Ackerman et al, in the year 1993. It is characterized by a heterogeneous clinical spectrum and a specific histopathological pattern. It has been described in association with a number of auto-immune disorders, drugs, fungal infections, and malignancies. It may manifest as papules, patches, plaques, nodules, annular lesions, or less frequently, as described classically, as indurated linear subcutaneous cords. This case is being reported as interstitial granulomatous dermatitis is rare dermatological manifestation of rheumatoid arthritis and presentation as large ulcerated skin lesions is further extremely rare. In this case report we describe a 67-year-old gentleman with Seropositive Rheumatoid arthritis. He presented to our hospital with complaints of multiple well defined skin lesions with pus discharge over lower limbs, trunk and upper limbs. Biopsies from left upper limb and abdomen showed focal necrobiosis with surrounding granulomatous inflammation with rare perivascular granulomas. He was subsequently diagnosed to have interstitial granulomatous dermatitis based on histopathological examination. Interstitial granulomatous dermatitis has been described to be associated with a number of autoimmune conditions and drugs. It is very important for clinicians to have knowledge about this rare skin condition as it may be heralding presentation of a serious underlying condition like lymphoproliferative disorders or solid organ malignancies. There is no specific treatment and causative disease has to be diagnosed and targeted.

Pioderma gangrenoso como manifestación Inicial de síndrome mielodisplásico / Pyoderma gangrenosum as initial manifestation of syndrome myelodysplastic

El pioderma gangrenoso es una dermatosis neutrofílica poco común que se presenta como un desorden inflamatorio y ulcerativo de la piel. Más de la mitad de los pacientes que desarrollan esta afección, tienen asociada una enfermedad sistémica subyacente. Caso clínico: paciente femenina de 79 años de edad, previamente sana, quien acude por presentar desde 4 días antes, flictenas violáceas, de contenido serohemático que se decapitan dejando úlcera superficial de fondo eritematoso, y luego profundizaron, no mejoraron con antibióticos por lo que se toma biopsia cutánea que reporta hallazgos compatibles con pioderma gangrenoso; se inició tratamiento con corticoides tópicos y sistémicos evidenciando mejoría de las lesiones. Durante la investigación etiológica se constató descenso de todas las líneas hematológicas y la biopsia de médula ósea mostró síndrome mielodisplásico con displasia unilínea de bajo grado. Discusión: El pioderma gangrenoso de tipo buloso, está comúnmente asociado a enfermedades hematológicas; en pacientes sin alteraciones hematológicas iniciales, debe haber un seguimiento estricto enfocado en la búsqueda de estos trastornos en un lapso de hasta 10 años. Se trata con fármacos inmunosupresores e inmunomoduladores. Conclusiones: ante la presencia de pioderma gangrenoso se debe sospechar síndrome mielodisplásico(AU)

Pyoderma gangrenosum is an uncommon neutrophilic dermatosis that presents as an inflammatory and ulcerative disorder of the skin. More than half of patients with pyoderma gangrenosum develop the disorder in association with an underlying systemic disease. Clinical case: 79-year-old female patient, previously healthy, who consulted for a period of 4 days, purplish skin lesions, which, once decapitated, showed a superficial ulcer that did not improve with broad spectrum antibiotics. The biopsy is taken, which reports findings compatible with pyoderma gangrenosum, so treatment with topical and systemic corticosteroids is started showing improvement of the lesions. During the diagnostic workup, pancytopenia was verified and, after ruling out other etiologies, bone marrow biopsy was performed, which allowed the diagnosis of lower-risk myelodysplastic syndrome with single lineage dysplasia. Discussion: Bullous pyoderma gangrenosum is most commonly seen in patients with hematologic disease; due to the strong association between bullous PG and hematologic disease, patients who present without an associated hematologic disorder should be followed closely for the development of a hematologic disorder. Conclusion: Etiological search for pyoderma gangrenosum allows early diagnosis and timely treatment of the underlying disease(AU)

Dermatosis neutrofílica de las manos y rostro posterior a mordedura de perro: reporte de caso y revisión de la literatura / Neutrophilic dermatosis of the hands and face after dog bite: case report and literature review

La Dermatosis neutrofílica de las manos es consi-derada una variante localizada acral del Síndrome de Sweet, más frecuente en mujeres y principal-mente asociada a enfermedades hematológicas. Las lesiones aparecen como pápulas, vesículas, nó-dulos, placas, úlceras y ampollas, principalmente en el dorso de las manos. Aproximadamente la mi-tad de los pacientes presenta fenómeno de patergia como factor desencadenante.En el presente caso clínico se describe una derma-tosis neutrofílica de las manos posterior a morde-dura de perro, asociado a mielofibrosis primaria y desarrollo de lesiones faciales.

Neutrophilic dermatosis of the hands is conside-red an acral localized variant of Sweet Syndrome, more frequent in women and mainly associated with hematological diseases. The lesions appear as papules, vesicles, nodules, plaques, ulcers, and blisters, mainly on the back of the hands. Appro-ximately half of the patients present a phenome-non of pathergy as a triggering factor. Herein we describe a case of neutrophilic dermatosis of the hands after a dog bite, associated with primary myelofibrosis and development of facial lesions.

A Case of Idiopathic Palmoplantar Eccrine Hidradenitis in Adult / 대한피부과학회지

No abstract available.

A Case of Immunoglobulin A Pemphigus: Intraepidermal Neutrophilic Dermatosis Type / 대한피부과학회지

Immunoglobulin A (IgA) pemphigus is a rare variant of an autoimmune bullous disease with IgA antibodies. IgA pemphigus is divided into 2 major subtypes: the subcorneal pustular dermatosis (SPD) type and intraepidermal neutrophilic (IEN) dermatosis type. We documented a case of an 18-year-old woman with recurrent generalized blisters and pustules that were especially severe in the intertriginous areas. Some half-and-half blisters and coalesced pustules in an annular pattern with crusts were simultaneously observed. A biopsy specimen from one of the half-and-half blister lesions showed intraepidermal separation with multiple neutrophils. Direct immunofluorescence staining revealed lace-like intercellular deposition of IgA in the entire epidermis. IgA antibody deposits were also observed in the patient's serum. The eruptions cleared with systemic steroids and colchicine 0.6 mg for 1 week, and the patient remained in partial remission at the 8-month follow-up. Herein, we report a case of IEN-type IgA pemphigus, clinically mimicking SPD with half-and-half blisters.