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1.
Rev. cuba. pediatr ; 952023. ilus
Article in Spanish | LILACS, CUMED | ID: biblio-1515283

ABSTRACT

Introducción: Los neumatoceles y las bulas pulmonares son lesiones que se observan en los niños casi siempre asociadas a neumonías infecciosas, aunque sus causas pueden ser diversas. La importancia clínica de estos procesos radica en el peligro de crecimiento progresivo, que puede comprometer las funciones respiratoria y cardiovascular. Objetivo: Describir las experiencias derivadas del proceso de diagnóstico por imágenes y del tratamiento invasivo de casos atendidos. Presentación de los casos: Desde finales de 2021 y durante un período de un año, se atendieron, en la unidad de cuidados intensivos pediátricos del Hospital Pediátrico Universitario de Cienfuegos, cinco niños con neumonías extensas, que desarrollaron bulas de gran tamaño varios días después del tratamiento antimicrobiano adecuado. Estas necesitaron drenaje y aspiración percutáneos debido a su magnitud y a la presencia de síntomas cardiovasculares. Conclusiones: Las bulas que aparecieron como complicación de la neumonía en el niño pueden presentarse con una frecuencia no despreciable, y hay que mantenerse atentos a su evolución, porque, a diferencia de los neumatoceles, pueden crecer progresivamente y comprometer las funciones respiratoria y cardiovascular. El drenaje percutáneo y aspiración continua por cinco días resultó un método seguro y eficaz para tratar estos procesos(AU)


Introduction: Pneumoatoceles and pulmonary bullae are lesions that are observed in children almost always associated with infectious pneumonia, although their causes may be diverse. The clinical importance of these processes lies in the danger of progressive growth, which can compromise respiratory and cardiovascular functions. Objective: To describe the experiences derived from the imaging process and the invasive treatment of treated cases. Presentation of the cases: Since the end of 2021 and for a period of one year, five children with extensive pneumonia were treated in the pediatric intensive care unit of the University Pediatric Hospital of Cienfuegos, who developed large bullae several days after appropriate antimicrobial treatment. The bullae required percutaneous drainage and aspiration due to their magnitude and the presence of cardiovascular symptoms. Conclusions: The bulla that appeared as a complication of pneumonia in the child can occur with a not negligible frequency, and it is necessary to be attentive to their evolution, because, unlike pneumoatoceles, can grow progressively and compromise respiratory and cardiovascular functions. Percutaneous drainage and continuous aspiration for five days was a safe and effective method to treat these processes(AU)


Subject(s)
Male , Female , Infant , Child, Preschool , Child , Pleural Effusion/drug therapy , Pneumonia/complications , Pneumonia/diagnostic imaging , Asthenia/etiology , Tachycardia/complications , Residence Characteristics , Blister/etiology , Back Pain , Cough , Thoracentesis/methods , COVID-19 , Thorax/diagnostic imaging , Ceftriaxone/therapeutic use , Vancomycin/therapeutic use , Drainage/instrumentation , Levofloxacin/therapeutic use , Anemia
2.
Rev. venez. cir ; 76(1): 65-71, 2023. tab, graf
Article in Spanish | LILACS, LIVECS | ID: biblio-1552965

ABSTRACT

Objetivo: Múltiples factores preoperatorios pueden complicar una Colecistectomía Laparoscópica electiva. El índice clínico de complejidad quirúrgica agrupa esos factores y les otorga un puntaje que permite estimar la complejidad quirúrgica, permitiendo optimizar el manejo clínico de la colelitiasis, por eso, este trabajo busca determinar el rendimiento diagnóstico del índice clínico de complejidad quirúrgica en paciente operados en el Centro Médico Docente la Trinidad, de Litiasis Vesicular por colecistectomía laparoscópica electiva, en el tiempo comprendido entre el 2020 y 2021. Métodos: Estudio observacional retrospectivo que, mediante la obtención de datos de 63 historias de pacientes, se calculó el Índice Clínico de Complejidad Quirúrgica y sus subíndices: clínico-quirúrgico, edad y comorbilidades; para comparar cada variable, subíndice y puntaje total con el tiempo quirúrgico. Se calcularon medidas de posición, tendencia central y dispersión. Resultados: Un mayor puntaje en el índice clínico de complejidad quirúrgica se encontró asociado a un tiempo quirúrgico superior a dos horas. El punto de corte para tiempo quirúrgico mayor a 120 minutos fue de 5.5 puntos con una Sensibilidad de 53% y especificidad del 81%. Conclusiones: Un puntaje superior a 5 en el Índice Clínico de Complejidad Quirúrgica nos podría permitir estimar qué colecistectomías laparoscópicas van a tener una duración mayor a 2 horas y en ese sentido permitiría optimizar los planes quirúrgicos(AU)


Objective: Multiple preoperative factors can complicate elective laparoscopic cholecystectomy. The clinical index of surgical complexity groups these factors and gives them a score that allows estimating the surgical complexity, allowing optimizing the clinical management of cholelithiasis, therefore, this work seeks to determine the diagnostic performance of the clinical index of surgical complexity in patients operated at the Centro Médico Docente la Trinidad, of Cholelithiasis by elective laparoscopic cholecystectomy, in the time between 2020 and 2021. Methods: Retrospective observational study that, by obtaining data from 63 patient records, the Clinical Index of Surgical Complexity and its sub-indexes: clinical-surgical, age and comorbidities were calculated to compare each variable, sub-index and total score with the surgical time. Measures of position, central tendency and dispersion were calculated. Results: A higher score in the clinical index of surgical complexity was found to be associated with a surgical time of more than two hours. The cutoff point for surgical time greater than 120 minutes was 5.5 points with a Sensitivity of 53% and specificity of 81%. Conclusions: A score higher than 5 in the Clinical Index of Surgical Complexity could allow us to estimate which laparoscopic cholecystectomies are going to have a duration longer than 2 hours and, in that sense, would allow us to optimize surgical plans(AU)


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Blister/pathology , Cholecystectomy, Laparoscopic , Lithiasis
3.
Rev. Ciênc. Méd. Biol. (Impr.) ; 20(4): 637-642, fev 11, 2022. tab, fig
Article in Portuguese | LILACS | ID: biblio-1359524

ABSTRACT

Introdução: a Epidermólise Bolhosa hereditária (EB) é uma doença rara, caracterizada por formações de bolhas na pele e nas mucosas que sofrem mínimos traumas ou aparecem espontaneamente. As principais divisões de EB são Epidermólise Bolhosa Simples, Epidermólise Bolhosa Juncional, Epidermólise Bolhosa Distrófica e a Síndrome de Kindler. As manifestações bucais geralmente encontradas nos pacientes com EB são as bolhas no epitélio oral, microstomia, anquiloglossia, doença periondontal, hipoplasia do esmalte, cárie dentária, atrofia da maxila e prognatismo mandibular, variando para cada tipo da doença. Objetivo: relatar as manifestações bucais dos portadores de Epidermólise Bolhosa residentes no município de Barra da Estiva. Metodologia: trata-se de um estudo transversal, quantitativo e descritivo que foi desenvolvido no município de Barra da Estiva ­ BA com a população de portadores de Epidermólise Bolhosa, através da realização de anamnese e exame clínico. Estatística descritiva foi utilizada para tabular os dados coletados. Resultados: foram analisados 5 portadores de Epidermólise Bolhosa, a maioria do sexo masculino (60%), com média de idade de 5,6 anos. As manifestações bucais encontradas foram bolhas orais (100%), anquiloglossia (100%), língua despapilada (100%), hipoplasia de esmalte (80%), cárie (40%) e abertura de boca limitada em média de 28,6 mm. Conclusão: os portadores de Epidermólise Bolhosa apresentaram manifestações orais características da doença, tendo como consequências maiores dificuldades para realizar higienização bucal e tratamento odontológico, sendo necessário conhecimento prévio dos cirurgiões-dentistas para o atendimento desses pacientes.


Introduction: inherited Epidermolysis Bullosa (EB) is a rare disease, characterized by blistering of the skin and mucous membranes that suffer minimal trauma or appear spontaneously. The main divisions of EB are Simple Epidermolysis Bullosa, Junctional Epidermolysis Bullosa, Dystrophic Epidermolysis Bullosa and Kindler Syndrome. The oral manifestations usually found in patients with EB are blisters in the oral epithelium, microstomy, ankyloglossia, periodontal disease, enamel hypoplasia, dental caries, atrophy of the jaw and mandibular prognathism, varying for each type of disease. Objective: to report the oral manifestations of Epidermolysis Bullosa patients living in the municipality of Barra da Estiva. Methods: cross-sectional study, quantitative and descriptive that was developed in the municipality of Barra da Estiva-BA with the population of people with Epidermolysis Bullosa, through anamnesis and clinical examination. Descriptive statistics was used to tabulate the data collected. Results: 5 children with Epidermolysis Bullosa were analyzed, most male (60%), with an average age of 5,6 years. Oral manifestations found were oral blisters (100%), anquiloglossia (100%), loss of papillae of the tongue (100%), enamel hypoplasia (80%), tooth decay (40%) and mouth opening limited to the average of 28.6 mm. Conclusion: epidermolysis Bullosa children presented oral manifestations common to the disease, leading to difficulties in performing oral hygiene and dental treatment, requiring prior knowledge of dental surgeons for the care of these patients.


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Oral Manifestations , Blister , Epidermolysis Bullosa , Dental Enamel Hypoplasia , Microstomia , Demography , Epidemiology, Descriptive , Evaluation Studies as Topic
4.
Chinese Journal of Burns ; (6): 538-548, 2022.
Article in Chinese | WPRIM | ID: wpr-940957

ABSTRACT

Objective: To assess the current situation of early treatment of partial-thickness burn wounds by professional burn medical staff in China, and to further promote the standardized early clinical treatment of partial-thickness burn wounds. Methods: A cross-sectional investigation was conducted. From November 2020 to February 2021, the self-designed questionnaire for the early treatment of partial-thickness burn wounds was published through the "questionnaire star" website and shared through WeChat to conduct a convenient sampling survey of domestic medical staff engaged in burn specialty who met the inclusion criteria. The number, region, and grade of the affiliated hospital, the age, gender, occupation, and seniority of the respondents were recorded. The respondents were divided into physician group and nurse group, senior group and junior group, eastern region group and non-eastern region group, primary and secondary hospital group and tertiary hospital group. Then the seniority, grade of the affiliated hospital, region of the affiliated hospital of the respondents in physician group and nurse group, conventional treatment of partial-thickness burn blisters, reasons for retaining vesicular skin, reasons for removing vesicular skin, and the conventional selection and optimal solution recommendation of topical drugs or dressings for partial-thickness burn wounds in the early stage of respondents in each of all the groups were recorded. Data were statistically analyzed with chi-square test. Results: The survey covered 31 provinces, municipalities, and autonomous regions in China (except for Hong Kong, Macau, and Taiwan regions). A total of 979 questionnaires were recovered, which were all valid. The 979 respondents came from 449 hospitals across the country, including 203 hospitals in the eastern region, 116 hospitals in the western region, 99 hospitals in the central region, and 31 hospitals in the northeast region, 348 tertiary hospitals, 79 secondary hospitals, and 22 primary hospitals. The age of the respondents was (39±10) years. There were 543 males and 436 females, 656 physicians and 323 nurses, 473 juniors and 506 seniors, 460 in the eastern regions and 519 in the non-eastern regions, 818 in tertiary hospitals and 161 in primary and secondary hospitals. There were statistically significant differences in the composition of different seniority in the respondents between physician group and nurse group (χ2=44.32, P<0.01), while there were no statistically significant differences in grade or region of the affiliated hospital of the respondents between physician group and nurse group (P>0.05). There were no statistically significant differences in the conventional treatment of partial-thickness burn blisters among respondents between different occupational groups, seniority groups, and region of the affiliated hospital groups (P>0.05).The respondents in different grade of the affiliated hospital groups differed significantly in the conventional treatment of partial-thickness burn blisters (χ2=6.24, P<0.05). Compared with respondents in nurse group, larger percentage of respondents in physician group chose to retain vesicular skin for protecting the wounds and providing a moist environment, and alleviating the pain of dressing change (with χ2 values of 21.22 and 19.96, respectively, P values below 0.01), and smaller percentage of respondents in physician group chose to retain vesicular skin for prevention of wound infection (χ2=23.55, P<0.01). The reasons for retaining vesicular skin of respondents between physician group and nurse group were similar in accelerating wound healing, alleviating pigmentation and scar hyperplasia post wound healing (P>0.05). Compared with respondents in junior group, larger percentage of respondents in senior group chose to retain vesicular skin for protecting the wounds and providing a moist environment and alleviating the pain of dressing change (with χ2 values of 10.36 and 4.60, respectively, P<0.05 or P<0.01), and smaller percentage of respondents in senior group chose to retain vesicular skin for prevention of wound infection (χ2=8.20, P<0.01). The reasons for retaining vesicular skin of respondents in senior group and junior group were similar in accelerating wound healing, alleviating pigmentation and scar hyperplasia post wound healing (P>0.05). The 5 reasons for the respondents between eastern region group and non-eastern region group, primary and secondary hospital group and tertiary hospital group chose to retain vesicular skin were all similar (P>0.05). Compared with those in physician group, significantly higher percentage of respondents in nurse group were in favor of the following 6 reasons for removing the vesicular skin, including convenience for using more ideal dressings to protect the wounds, prevention of wound infection, facilitating the effect of topical drugs on the wounds, the likely rupture of blisters and wound contamination, accelerating wound healing, and alleviating pigmentation and scar hyperplasia post wound healing (with χ2 values of 4.35, 25.59, 11.83, 16.76, 46.31, and 17.54, respectively, P<0.05 or P<0.01). Compared with respondents in senior group, larger percentage of respondents in junior group chose to remove vesicular skin for the reasons such as the likely blister rupture and wound contamination, preventing wound infection, accelerating wound healing, and alleviating pigmentation and scar hyperplasia post wound healing (with χ2 values of 17.25, 18.63, 14.83, and 10.23, respectively, P values below 0.01). Compared with respondents in non-eastern region group, larger percentage of respondents in eastern region group chose to remove vesicular skin for preventing wound infection and the likely rupture of blisters and wound contamination (with χ2 values of 9.30 and 8.65, respectively, P values below 0.01). The 6 reasons for the respondents between tertiary hospital group and primary and secondary hospital group choose to remove vesicular skin were similar (P>0.05). Compared with respondents in physician group, larger percentage of respondents in nurse group chose to use moisturizing materials for partial-thickness burn wounds in the early stage (χ2=6.18, P<0.05), and smaller percentage of respondents in nurse group chose other topical drugs or dressings (χ2=5.20, P<0.05). Compared with respondents in junior group, larger percentage of respondents in senior group chose to use moisturizing materials and other topical drugs or dressings for partial-thickness burn wounds in the early stage (with χ2 values of 4.97 and 21.80, respectively, P<0.05 or P<0.01). Compared with respondents in non-eastern region group, larger percentage of respondents in eastern region group chose to use topical antimicrobial drugs for partial-thickness burn wounds in the early stage (χ2=4.09, P<0.05), and smaller percentage of respondents in eastern region group chose to use other topical drugs or dressings for the partial-thickness burn wounds in the early stage (χ2=5.63, P<0.05). Compared with respondents in primary and secondary hospital group, larger percentage of respondents in tertiary hospital group chose to use biological dressings for partial-thickness burn wounds in the early stage (χ2=9.38, P<0.01). The optimal solution recommendation of topical drugs or dressings for partial-thickness burn wounds in the early stage varied significantly among the respondents between different occupational groups and seniority groups (with χ2 values of 39.58 and 19.93, respectively, P values below 0.01). There were no statistically significant differences between eastern and non-eastern region groups, tertiary hospital group and primary and secondary hospital groups in optimal solution recommendation of topical drugs or dressings for partial-thickness burn wounds in the early stage (P>0.05). Conclusions: The conventional treatment measures of partial-thickness burn blisters and reasons for preserving blister skin by professional burn medical staff in China are relatively consistent, but there are great differences in the selection of reasons for removing blister skin, the conventional selection and optimal solution recommendation of topical drugs or dressings for partial-thickness burn wounds in the early stage. Therefore, it is urgent to establish a clinical treatment standard for partial-thickness burn wounds.


Subject(s)
Adult , Female , Humans , Male , Middle Aged , Blister , Burns/drug therapy , Cicatrix/pathology , Cross-Sectional Studies , Hyperplasia , Medical Staff , Occupations , Pain , Soft Tissue Injuries , Wound Infection
5.
Chinese Journal of Industrial Hygiene and Occupational Diseases ; (12): 305-307, 2022.
Article in Chinese | WPRIM | ID: wpr-935799

ABSTRACT

Allyl alcohol is an intermediate widely used in industrial production activities, which has caused many occupational damage in China. In this paper, the data of a case of skin damage and clinical cure of inhalation pneumonia caused by occupational allyl alcohol exposure were analyzed and summarized. The patient accidentally inhaled large amounts of allyl alcohol at work and had skin exposure, which was mainly manifested as aspiration pneumonia, respiratory failure, eye damage and skin bullae. After short-term hormone shock therapy, mechanical ventilation, infection prevention, local treatment and other comprehensive treatment, the patient was clinically cured. This case suggests that chemical pneumonia and respiratory failure may be caused by inhalation of allyl alcohol, and skin bullae may be caused by skin contact. Early treatment and hormone use should be emphasized.


Subject(s)
Humans , Blister , Hormones , Pneumonia , Propanols , Respiratory Insufficiency
6.
Acta Academiae Medicinae Sinicae ; (6): 227-235, 2022.
Article in Chinese | WPRIM | ID: wpr-927870

ABSTRACT

Objective To investigate the clinical characteristics and genetic mutations in Kindler syndrome(KS)and provide a theoretical basis for the diagnosis and treatment of KS. Methods The clinical data of one case of KS from Peking Union Medical College Hospital and 185 cases reported in literature were collected. The gene mutation types,patient clinical data,and tumor characteristics were statistically analyzed. Results A total of 186 cases were enrolled,including 110 males and 76 females,with the mean age of(28±16)years. The data of gene mutation and specific clinical manifestations were available in 151 and 94 patients,respectively. The main clinical manifestations of KS included poikiloderma,occurrence of blister in childhood,and photosensitivity,and the secondary clinical manifestations included oral inflammation,palmoplantar keratoderma,webbing/pseudoainhum,dysphagia,urethral stricture and so on.Oral inflammation(r=0.234,P=0.023),palmoplantar keratoderma(r=0.325,P=0.001),webbing/pseudoainhum(r=0.247,P=0.016),dysphagia(r=0.333,P=0.001),urethral stricture(r=0.280,P=0.006)were significantly correlated with age,showing significantly higher incidence in the patients over 32 years old.Urethral stricture(χ2=11.292,P=0.001)and anal stenosis(χ2=4.014,P=0.045)were significantly correlated with sex,with higher incidence in males.Eighty different mutations were found in 151 patients,and the most common gene mutation was c.676C>T.Forty-one tumors occurred in 27 patients,among which squamous cell carcinoma accounted for 92.7%. The gene mutation site had no significant correlation with squamous cell carcinoma or patient country. Conclusions The c.676C>T in FERMT1 gene is the most common mutation in KS.The patients are prone to squamous cell carcinoma and mainly attacked at the exposure sites(hand and mouth).


Subject(s)
Adolescent , Adult , Child , Female , Humans , Male , Young Adult , Ainhum , Blister , Carcinoma, Squamous Cell , Constriction, Pathologic , Deglutition Disorders/complications , Epidermolysis Bullosa , Inflammation , Keratoderma, Palmoplantar/complications , Membrane Proteins , Mutation , Neoplasm Proteins/genetics , Periodontal Diseases , Photosensitivity Disorders , Urethral Stricture/complications
7.
Diagn. tratamento ; 26(4): 140-3, out-dez. 2021. ilus, ilus, tab
Article in Portuguese | LILACS | ID: biblio-1348608

ABSTRACT

Contexto: As reações hansênicas são episódios inflamatórios agudos imunomediados que podem ocorrer em indivíduos com hanseníase. Lesões atípicas de eritema nodoso hansênico (ENH) são raras e podem se apresentar como pústulas, bolhas, necrose, úlceras e, eventualmente, podem simular outras doenças como a síndrome de Sweet e eritema multiforme. Descrição do caso: Paciente do sexo masculino, 24 anos de idade, com diagnóstico prévio de hanseníase virchowiana e realização de tratamento adequado, com término da poliquimioterapia quatro anos antes de comparecer ao nosso serviço quando foi avaliado, apresentando pequenas vesículas translúcidas agrupadas sobre uma base eritematosa e áreas com crostas necróticas, simulando lesões herpéticas, na porção superior das costas, parte inferior do pescoço e haste do pênis. O exame histopatológico da biópsia cutânea foi sugestivo de reação tipo 2 e o tratamento com talidomida foi satisfatório. Discussão: As reações na hanseníase representam episódios de hipersensibilidade aguda a antígenos de Mycobacterium leprae e podem se desenvolver antes, durante ou após o término do tratamento. Acredita-se que o ENH, ou reações do tipo 2, ocorram devido à deposição de imunocomplexos em múltiplos órgãos em pacientes que apresentam a forma multibacilar. Conclusões: As reações hansênicas são desafios no manejo da hanseníase, especialmente pelo fato de a maioria das sequelas permanentes ser secundária a esses episódios inflamatórios agudos. O conhecimento das possíveis apresentações atípicas das reações hansênicas é fundamental para o diagnóstico precoce e o tratamento adequado, a fim de prevenir morbidade grave relacionada à hanseníase.


Subject(s)
Humans , Male , Adult , Leprosy, Lepromatous , Blister , Leprosy, Multibacillary , Herpes Simplex , Leprosy
8.
Arch. pediatr. Urug ; 92(2): e307, dic. 2021. ilus, tab
Article in Spanish | LILACS, UY-BNMED, BNUY | ID: biblio-1339135

ABSTRACT

Las porfirias son un grupo complejo y heterogéneo de defectos en la vía de la síntesis del hemo. La porfiria hepato eritropoyética es un subtipo muy poco frecuente y de presentación en la infancia, con compromiso cutáneo predominante. Describimos el caso clínico de una paciente de 5 años, que se presenta con lesiones cutáneas e hipertricosis, se confirma el diagnóstico por elevación de uroporfirinas en orina y secuenciación del gen UROD.


Porphyria is a complex and heterogeneous group of heme synthesis disorder. Hepato-erythropoietic porphyria is a very rare subtype that onsets in childhood, and shows predominant skin involvement. We describe the clinical case of a 5-year-old patient who showed skin lesions and hypertrichosis and whose diagnosis was confirmed due to increased uroporphyrins in urine and UROD gene sequencing


A porfiria é um grupo complexo e heterogêneo de distúrbios da síntese do grupo heme. A porfiria hepato-eritropoiética é um subtipo muito raro que se inicia na infância e mostra envolvimento predominante da pele. Descrevemos o caso clínico de uma paciente de 5 anos que apresentou lesões cutâneas e hipertricose e cujo diagnóstico foi confirmado por aumento de uroporfirinas na urina e sequenciamento do gene UROD.


Subject(s)
Humans , Female , Child, Preschool , Blister/etiology , Porphyria, Hepatoerythropoietic/complications , Porphyria, Hepatoerythropoietic/genetics , Porphyria, Hepatoerythropoietic/urine , Diabetes Mellitus, Type 1/complications , Hypertrichosis/etiology , Uroporphyrinogen Decarboxylase/analysis , Uroporphyrins/urine , Blister/drug therapy , Coproporphyrins/urine , Hypertrichosis/drug therapy
9.
Rev. MED ; 29(2): 121-126, jul.-dic. 2021. graf
Article in Spanish | LILACS, COLNAL | ID: biblio-1422809

ABSTRACT

Resumen: la epidermólisis bullosa (EB) es un grupo heterogéneo de enfermedades mecanobulosas hereditarias caracterizadas por diversos grados de fragilidad cutánea y mucosa causada por mutaciones que afectan a las proteínas estructurales de la piel 1,2,3. Como consecuencia de estas se forman ampollas y vesículas tras mínimos traumatismos o fuerzas de fricción, con afectación variable de otros órganos. Por ser una condición poco común, el presente artículo expone el caso de una niña de cuatro años con diagnóstico de epidermólisis bullosa. El reporte de caso se realizó a través de la observación de un fenómeno, bajo la metodología de estudio de caso, analizando variables clínicas, de laboratorio e histopatológicas. Los hallazgos físicos encontrados en la paciente coinciden con lo reportado por Torres-Ibérico et al. 8, donde predominan la aparición de ampollas. Para el diagnóstico de la enfermedad en este caso se hizo necesario realizar una biopsia de piel. Como conclusión, se recomienda un abordaje multidisciplinar en la intervención del paciente. Lo que se fundamenta en la protección de los tejidos potencialmente sometidos a lesiones, la aplicación de sofisticados vendajes, un generoso soporte nutricional y, de ser posible, un tratamiento temprano o intervenciones quirúrgicas para corregir las complicaciones extra cutáneas.


Abstract: epidermolysis bullosa (EB) Is a heterogeneous group of Inherited mechanobullous diseases characterized by varying degrees of cutaneous and mucosal fragility caused by mutations affecting structural skin proteins 1,2,3. As a consequence of these, blisters and vesicles are formed after minimal trauma or friction forces, with variable involvement of other organs. As it is a rare condition, this article presentsthe case of a four-year-old girl diagnosed with epidermolysis bullosa. The case report was made by means ofthe observation of a phenomenon, under the case study methodology, analyzing clinical, laboratory and histopathologlcal variables. The physical findings found In the patient coincide with those reported by Torres-Iberlco et al. 8, where the appearance of blisters predominates. For the diagnosis ofthe disease In this case It was necessary to perform a skin biopsy. In conclusion, a multidisciplinary approach to patient Intervention is recommended. This is based on the protection of tissues potentially subject to injury, the application of sophisticated bandages, generous nutritional support and, If possible, early treatment or surgical interventions to correct extracutaneous complications.


Resumo: epidermólise bolhosa (EB) é um grupo heterogêneo de doenças mecanobulares hereditárias caracterizadas por graus variados de fragilidade cutânea e mucosa causada por mutações que afetam proteínas estruturais da pele 1,2,3. Como consequência disso, bolhas e vesículas são formadas após trauma mínimo ou forças de atrito, com envolvimento variável de outros órgãos. Por ser urna condão rara, este artigo apresenta o caso de urna menina de quatro anos diagnosticada com epidermólise bolhosa. O relato de caso foi feito por meio da observação de um fenómeno, sob a metodologia de estudo de caso, analisando variáveis clínicas, laboratoriais e histopatológicas. Os achados físicos encontrados no paciente coincidem com os relatados por Torres-Iberico et al. 8, onde predomina o aparecimento de bolhas. Para o diagnóstico da doença neste caso foi necessário realizar urna biópsia de pele. Como conclusão, recomenda-se urna abordagem multidisciplinar na Intervenção do paciente. Isso se baseia na proteção dos tecidos potencialmente sujeitos a lesões, na aplicação de curativos sofisticados, no suporte nutricional generoso e, se possível, no tratamento precoce ou ¡intervenções cirúrgicas para corrigir complicações extracutâneas.


Subject(s)
Humans , Female , Child, Preschool , Blister/drug therapy
10.
Dermatol. argent ; 27(4): 133-143, oct. - dic. 2021. il, tab
Article in Spanish | LILACS, BINACIS | ID: biblio-1381133

ABSTRACT

El sistema del complemento es un conjunto de proteínas asociadas a la regulación de la inmunidad y a la protección del huésped. Existen tres vías de activación llamadas clásica, alternativa y asociada a las lectinas, que culminan en la producción de opsoninas, de anafilotoxinas y del complejo de ataque a la membrana. La activación del sistema del complemento es fundamental en la defensa cutánea contra agentes microbiológicos, como también en la regulación de la inflamación y de la lesión tisular. En diversas enfermedades cutáneas puede constatarse hiperactividad, deficiencia o anomalías en el control del sistema del complemento. Mediante mecanismos autoinmunitarios con depósito de anticuerpos, o por efecto citotóxico sobre la epidermis o las células vasculares, se observa un efecto inflamatorio directo como ocurre en el lupus eritematoso sistémico o en las enfermedades ampollares autoinmunes. Además, las deficiencias en la regulación del sistema del complemento generan la activación de vías colaterales proinflamatorias como en el caso del sistema calicreínacinina (quinina) en el angioedema hereditario. En este trabajo se describe la fisiología del sistema del complemento, su relevancia en algunas patologías cutáneas frecuentes y las alteraciones en los estudios de laboratorio.


The complement system is a set of proteins associated with the regulation of immunity and the host protection. There are three activation pathways called classical, alternative, and lectin-associated, which culminate in the production of opsonins, anaphylatoxins, and the membrane attack complex. The activation of the complement system plays a fundamental role in the cutaneous defense against microbiological agents, as well as in the regulation of inflammation and tissue injury. In severalskin diseases, hyperactivity, deficiency or abnormalities in the control of the complement system can be observed. Through autoimmune mechanisms with antibody deposition or by cytotoxic effect on the epidermis or vascular cells, a direct inflammatory effect is observed, as occurs in systemic lupus erythematosus or in autoimmune bullous diseases. Moreover, deficiencies in the regulation of the complement system lead to the activation of pro-inflammatory collateral pathways, as in the case of the kallikrein-kinin system in hereditary angioedema. In this manuscript, we describe the physiology of the complement system, its relevance in common skin pathologies, and alterations in laboratory studies.


Subject(s)
Humans , Male , Female , Skin Diseases/pathology , Complement System Proteins/physiology , Psoriasis , Complement Activating Enzymes , Skin Diseases, Vesiculobullous , Blister , Angioedemas, Hereditary
11.
Rev. argent. cir ; 113(1): 121-124, abr. 2021. graf
Article in Spanish | LILACS, BINACIS | ID: biblio-1288182

ABSTRACT

RESUMEN La neoplasia papilar intracolecística con carcinoma mucinoso invasor y células en anillo de sello es una variedad de cáncer de vesícula, una patología agresiva y con mal pronóstico, la cual puede presentarse en forma similar a una colecistitis y, a pesar del tratamiento operatorio y quimioterapia posterior, la supervivencia y pronóstico son peores en relación con los otros cánceres de vesícula. Este artículo tie ne por objetivo describir un tipo histológico muy específico, de baja frecuencia, de cáncer de vesícula y el tratamiento realizado.


ABSTRACT Intracholecystic papillary neoplasm with invasive mucinous adenocarcinoma and signet ring cells is a rare, aggressive variety of gallbladder cancer, with symptoms mimicking cholecystitis. Survival and prognosis are worse that other types of gallbladder cancer despite surgery and chemotherapy. The aim of this article is to describe a case of a rare gallbladder cancer with specific histology and the treatment performed


Subject(s)
Humans , Male , Aged , Gallbladder Neoplasms , Pathology , Survival , Therapeutics , Grief , Cells , Baja , Blister , Drug Therapy , Gallbladder , Histology , Neoplasms
13.
Prensa méd. argent ; 107(1): 13-17, 20210000. fig
Article in Spanish | LILACS, BINACIS | ID: biblio-1362062

ABSTRACT

La agenesia de la vesícula biliar (AVB) es una entidad congénita rara. La mayoría de los pacientes, permanecen asintomáticos, mientras que los que presentan síntomas informan síntomas que imitan el cólico biliar. La evaluación inicial para la sospecha de patología de la vesícula biliar, como la ecografía del cuadrante superior derecho, Puede ser engañosa o no concluyente. En consecuencia, algunos pacientes son finalmente diagnosticados intra-operatoriamente. Por lo tanto, deben mantener la AVB como un diagnóstico diferencial y deben realizarse como la colangiopancreatografía por resonancia magnética (CPRMN) cuando otras pruebas no sean concluyentes. Presentamos una mujer de 39 años que presento síntomas crónicos compatibles con cólico biliar y una ecografía equívoca informada como escleroatrófica con colelitiasis. Se le realizó laparoscopia durante la cual se constató la ausencia de vesícula biliar. La CPRM posoperatoria confirmó el diagnóstico de AG.


Gallbladder agenesis (GA) is a rare congenital entity. Most patients remain asymptomatic, while those with symptoms report symptoms that mimic bile colic. Initial evaluation for suspected gallbladder pathology, such as ultrasound of the right upper quadrant, May be misleading or inconclusive. As a result, some patients are eventually diagnosed intra-operatively. Therefore, GA should be maintained as a differential diagnosis and should be performed as magnetic resonance cholangiopancreatography (MRI) when other tests are inconclusive. We present a 39-year-old woman who has chronic symptoms compatible with biliary colic and an equivocal ultrasound reported as scleroatrofic with cholelithiasis. Laparoscopy was performed during which the absence of gallbladder was found. Postoperative CPRM confirmed the diagnosis of GA.


Subject(s)
Humans , Female , Adult , Congenital Abnormalities/pathology , Biliary Tract/abnormalities , Blister/pathology , Laparoscopy , Cholangiopancreatography, Magnetic Resonance , Diagnosis, Differential
14.
An. bras. dermatol ; 96(2): 155-162, Mar.-Apr. 2021. tab, graf
Article in English | LILACS | ID: biblio-1248738

ABSTRACT

Abstract Background: Epidermolysis bullosa is characterized by cutaneous fragility and blistering. Historically, diagnosis is achieved by immunofluorescence mapping or transmission electron microscopy, both involving biopsy procedures. Genetic analysis, especially through next-generation sequencing, is an important tool for the diagnosis of this disease. In Brazil, access to diagnostic methods is limited, and consequently, most patients do not have an accurate diagnosis. Diagnosis allows the indication of prognosis and genetic counselling of the patient. Objectives: To evaluate the cost-effectiveness of a gene panel compared to immunofluorescence mapping and transmission electron microscopy by analyzing its benefits, limitations, and economic aspects. Methods: The gene panel included the 11 main genes associated with epidermolysis bullosa. The techniques were compared, assessing the average cost, advantages, and limitations, through a price survey and literature review. Results: Both immunofluorescence mapping and transmission electron microscopy require skin biopsy, are dependent on the investigator's expertise, and are subject to frequent inconclusive results. The gene panel is effective for the conclusive diagnosis of epidermolysis bullosa, presents high efficiency and accuracy, is economically feasible, and excludes the need for biopsy. The gene panel allows for prognosis, prenatal genetic diagnosis, and genetic counseling. Study limitations: It was not possible to find laboratories that perform transmission electron microscopy for epidermolysis bullosa diagnosis in Brazil. Conclusion: This study supports the gene panel as the first-choice method for epidermolysis bullosa diagnosis.


Subject(s)
Humans , Epidermolysis Bullosa/diagnosis , Epidermolysis Bullosa/genetics , Skin , Biopsy , Brazil , Blister
15.
Infectio ; 25(1): 45-48, ene.-mar. 2021. tab, graf
Article in Spanish | LILACS, COLNAL | ID: biblio-1154401

ABSTRACT

Resumen La vasculitis leucocitoclastica es una patologìa que compromete los vasos pequeños y cuya causa predominantemente se ha descrito como idiopatica. Se presenta el caso de una mujer de 78 años hipertensa, diabética y con enfermedad renal crónica en estadio 5, que presentó lesiones limitadas a la piel posterior a la administración de oxacilina para manejo de bacteremia por SAMS. La presentación clínica se basó en purpuras palpables predominantemente en miembros inferiores y lesiones dolorosas coalescentes que formaban ampollas de contenido hemorrágico. Estas lesiones resolvieron gradualmente después del cambio de la terapia mencionada anteriormente. La biopsia fue compatible con vasculitis leucocitoclástica, con paraclínicos que descartaron causas infecciosas y autoinmunes.


Abstract Leukocytoclastic vasculitis is a pathology that involves small vessels and whose cause has been predominantly described as idiopathic. The clinical case of a 78-year-old woman with hypertension, diabetic and chronic stage 5 kidney disease, who presented limited skin lesions after administration of oxacillin for management of bacteremia by MSSA. The clinical presentation consisted on palpable purpura predominantly in the lower limbs and painful coalescent lesions that formed blisters of hemorrhagic content. Lesions gradually resolved after the change of the therapy mentioned above. The biopsy was compatible with leukocytocastic vasculitis, with paraclinics who ruled out infectious and autoimmune causes.


Subject(s)
Humans , Male , Aged , Vasculitis, Leukocytoclastic, Cutaneous , Oxacillin , Blister , Renal Insufficiency, Chronic , Kidney Diseases
16.
Rev. colomb. radiol ; 32(4): 5363-5365, dic. 2021. imag
Article in English, Spanish | LILACS, COLNAL | ID: biblio-1428681

ABSTRACT

Las malformaciones congénitas de la vesícula son variadas y algunas de ellas muy raras, como es el caso de la vesícula tabicada. Esta no es una patología frecuente, generalmente es un hallazgo imagenológico incidental en pacientes con dolor abdominal crónico, la mayoría de ellos adultos mayores, por lo que es importante conocerla y reconocerla. Se puede diagnosticar con todos los métodos de imagen, desde la ecografía hasta la resonancia magnética, por lo cual cobra importancia para los profesionales de esta rama tener conocimiento claro de sus manifestaciones.


Congenital malformations of the gallbladder are varied and some of them are very rare, as is the case of the septal gallbladder. This is not a frequent pathology, it is usually an incidental imaging finding in patients with chronic abdominal pain, most of them older adults, so it is important to know and recognize it. It can be diagnosed with all imaging methods from ultrasound to magnetic resonance, which is why it is important for professionals in this branch to know and recognize it.


Subject(s)
Abdominal Pain , Blister , Multidetector Computed Tomography
17.
An. bras. dermatol ; 95(5): 551-569, Sept.-Oct. 2020. tab, graf
Article in English | LILACS, ColecionaSUS | ID: biblio-1130935

ABSTRACT

Abstract Inherited epidermolysis bullosa is a group of genetic diseases characterized by skin fragility and blistering on the skin and mucous membranes in response to minimal trauma. Epidermolysis bullosa is clinically and genetically very heterogeneous, being classified into four main types according to the layer of skin in which blistering occurs: epidermolysis bullosa simplex (intraepidermal), junctional epidermolysis bullosa (within the lamina lucida of the basement membrane), dystrophic epidermolysis bullosa (below the basement membrane), and Kindler epidermolysis bullosa (mixed skin cleavage pattern). Furthermore, epidermolysis bullosa is stratified into several subtypes, which consider the clinical characteristics, the distribution of the blisters, and the severity of cutaneous and extracutaneous signs. Pathogenic variants in at least 16 genes that encode proteins essential for the integrity and adhesion of skin layers have already been associated with different subtypes of epidermolysis bullosa. The marked heterogeneity of the disease, which includes phenotypes with a broad spectrum of severity and many causal genes, hinders its classification and diagnosis. For this reason, dermatologists and geneticists regularly review and update the classification criteria. This review aimed to update the state of the art on inherited epidermolysis bullosa, with a special focus on the associated clinical and genetic aspects, presenting data from the most recent reclassification consensus, published in 2020.


Subject(s)
Humans , Epidermolysis Bullosa Dystrophica/genetics , Epidermolysis Bullosa, Junctional/genetics , Epidermolysis Bullosa/genetics , Skin , Blister
18.
An. bras. dermatol ; 95(4): 514-517, July-Aug. 2020. graf
Article in English | LILACS, ColecionaSUS | ID: biblio-1130912

ABSTRACT

Abstract Dissecting cellulitis is a chronic, progressive, and relapsing inflammatory disease that predominantly affects the vertex and occiput of young Afro-descendent men. It starts with papules and pustules that evolve to nodules, abscesses, and cicatricial alopecia. This article illustrates the evolutive trichoscopy of dissecting cellulitis, from its early phase, through the abscess phase, to the fibrotic cicatricial phase. Trichoscopy complements clinical-pathological classification, representing a complementary tool useful in early diagnosis and monitoring of the patient during treatment.


Subject(s)
Humans , Male , Cellulitis , Dermoscopy , Recurrence , Blister , Alopecia
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