RESUMO
This interdisciplinary paper summarizes the news in the diagnosis and treatment of chronic urticaria (CU), and provides concepts, definitions and evidence-based suggestions for its management. Urticaria occurs in at least 20% of the population at some point in their lives. Acute urticaria (less than 6 weeks' duration), differs from CU in its etiology, but the onset of this disease is always acute. CU may occur as spontaneous (SCU) or induced (ICU). The diagnosis is simple, although a careful evaluation is necessary for differential diagnosis. ICU's diagnosis is mainly clinical, even if provocation tests can be useful. Supplementary studies should be limited and based on the clinical suspicion. Treatment may be divided into three approaches: avoidance, elimination or treatment of the cause, and pharmacological treatment. Recently treatment has been modified with the use of second-generation antihistamines as first-line and increased doses of nonsedating H1 antihistamines, up to 4 times, as second line. Antihistamines are essential to treat CU; however, 40% of patients do not achieve good control despite increased doses and require additional treatment. The most recent evidence indicates a group of drugs to be used as third line in these cases, to improve quality of life and to limit toxicity from frequent or chronic use of systemic steroids. Only 3 drugs are recommended as third line: omalizumab, cyclosporin A or anti-leukotrienes.
Assuntos
Antialérgicos/uso terapêutico , Antagonistas dos Receptores Histamínicos/uso terapêutico , Urticária/diagnóstico , Urticária/tratamento farmacológico , Urticária/etiologia , Algoritmos , Angioedema/tratamento farmacológico , Angioedema/patologia , Anticorpos Anti-Idiotípicos/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Argentina , Doenças Autoimunes/complicações , Doença Crônica , Ensaios Clínicos como Assunto , Ciclosporina/uso terapêutico , Diagnóstico Diferencial , Medicina Baseada em Evidências/economia , Humanos , Imunoglobulina E/metabolismo , Antagonistas de Leucotrienos/uso terapêutico , Omalizumab , Qualidade de Vida , Urticária/classificação , Urticária/complicações , Urticária/fisiopatologiaRESUMO
Chromoblastomycosis is a mutilating infection of the skin and subcutaneous tissues caused by melanized fungi belonging to the order Chaetothyriales. Proven cases of the main agent, Fonsecaea pedrosoi are mainly limited to (sub)tropical, humid climates of Latin and Central America and the Caribbean. Fonsecaea monophora has a global distribution along the equator. Cases outside the (sub)tropics have thus far mostly been considered to have been imported, but here we report the first endemic case by F. monophora from Argentina. Patient was a 82-year-old rural female worker from Corrientes, a province with a dry continental climate.
RESUMO
BACKGROUND: Dermatitis herpetiformis (DH), a well-established gluten-sensitive skin disorder presenting variable degrees of enteropathy, constitutes a very useful model in order to assess the utility of the celiac disease (CD)-related serology in patients with mild intestinal damage. OBJECTIVE: Our aim was to explore comparatively the performance of a panel of CD-related serologic tests in patients with DH. METHODS: We assessed a series of 18 consecutive patients with skin biopsy proven DH presenting the overall spectrum of intestinal damage ranging from normal mucosa (n = 6) to total villous atrophy (TVA) (n = 6) through partial villous atrophy (PVA) (n = 6). Sera were obtained from all patients while consuming a gluten containing diet. Serologic tests were antiendomysial, anti-tissue transglutaminase and antigliadin antibodies, and newly developed tests detecting both antibody isotypes (IgA and IgG) against deamidated synthetic gliadin-derived peptides (a-GDP). RESULTS: Serologic tests had a variable behaviour depending on the degree of enteropathy. While the majority of tests detected patients with TVA, only 50% of those with normal histology had positive assays. Patients with PVA had discordant results. Classical CD-specific tests were positive in only some patients with mild damage while all of them were identifed by a single assay detecting both isotypes of a-GDR CONCLUSION: The detection of a-GDP antibodies was the most reliable tool in order to identify gluten sensitivity in DH patients presenting a wide range of intestinal damage. Further studies should explore if these findings can be extrapolated to patients with CD having mild enteropathy.
Assuntos
Autoanticorpos/sangue , Doença Celíaca/diagnóstico , Dermatite Herpetiforme/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Atrofia , Biomarcadores/sangue , Biópsia , Doença Celíaca/imunologia , Doença Celíaca/patologia , Dermatite Herpetiforme/imunologia , Dermatite Herpetiforme/patologia , Feminino , Gliadina/imunologia , Humanos , Imunoglobulina A/sangue , Imunoglobulina G/sangue , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Índice de Gravidade de Doença , Transglutaminases/imunologiaRESUMO
Introducción: el penfigoide de las mucosas (PM), antes llamado mucoso, cicatrizal o mucosinequiante, representa un grupo heterogéneo de enfermedades ampollares autoinmunes inflamatorias crónicas que comprometen las mucosas o la piel, con tendencia a dejar secuelas cicatrizales. Existen autoanticuerpos contra distintos componentes de la zona de la membrana basal (BPAG1, BPAG2, integrina α6ß4, laminina 332, colágeno VII, entre otros), por lo que la inmunofluorescencia directa (IFD) es de suma importancia, así como la clínica, para su diagnóstico.Objetivo: realizar una revisión de los casos de PM diagnosticados durante un período de 24 años (enero de 1997- marzo de 2021) en el Sector de Enfermedades Ampollares del Hospital Ramos Mejía para determinar la epidemiología, la clínica y la terapéutica de esta enfermedad.Diseño: estudio retrospectivo descriptivo y observacional, en el que se analizaron las características clínicas e inmunopatológicas de 34 pacientes con diagnóstico de PM atendidos en el Servicio de Dermatología del Hospital Ramos Mejía desde enero de 1997 hasta marzo de 2021. Materiales y métodos: mediante las historias clínicas y los regis-tros iconográficos, se evaluaron las siguientes variables: prevalencia del diagnóstico de PM en los pacientes atendidos en el Sector, sexo, edad, antecedentes personales, mucosas afectadas, tiempo de evolución hasta el diagnóstico, hallazgos en la IFD, seguimiento clínico y tratamientos instaurados. Resultados: se estudió la evolución clínica de 34 pacientes diagnosticados con PM (5,3% del total de pacientes evaluados en el Sector de Patologías Ampollares). El sexo más afectado fue el femenino y la edad promedio en el momento del diagnóstico fue de 64 años. El 70,6% de los pacientes presentaron comorbilidades asociadas como hipertensión e hipotiroidismo. La mayoría refirió algún evento emocional como factor desencadenante. El sitio más comprometido fue la mucosa ocular y la cavidad oral fue la segunda en frecuencia. El tiempo de evolución promedio hasta el momento del diagnóstico fue de 4 años y 11 meses. El hallazgo más frecuente en la IFD fue la IgG lineal. El 17,6% de los pacientes interrumpieron el seguimiento clínico. El tratamiento más utilizado fue el mofetil micofenolato, con el que se obtuvo buena respuesta terapéutica. Conclusiones: el PM es una enfermedad autoinmune infrecuente que compromete las mucosas y, ocasionalmente, la piel. En este estudio, se observó que la principal mucosa afectada fue la conjuntival, a diferencia de lo referido en la bibliografía internacional dermatológica. El diagnóstico interdisciplinario temprano es fundamental para evitar las secuelas irreversibles.
Introduction: mucous membrane pemphigoid (MMP), also known as benign mucous membrane pemphigoid, cicatricial or mucosynechial pemphigoid, belongs to an heterogeneous group of chronic inflammatory autoimmune blistering diseases, which involves the mucous membranes (oral, ocular, pharyngeal, nasal, esophageal, laryngeal and anogenital) and/ or skin with tendency to scar formation. There are autoantibodies against different components of the basement membrane zone (BPAG 1- BPAG2, Integrin α6ß4, Laminin 332, Col VII, among others). The direct immunofluorescence (DIF) will be of paramount importance, as well as the clinical diagnosis.Objective: review the cases diagnosed with mucous membrane pemphigoid for 24 years (January 1997- March 2021) in the Blistering Disease Clinic at the Dermatology Department at the Ramos Mejia Hospital to establish the epidemiologic, clinic presentation and available treatments in this pathology.Design: retrospective descriptive and observational study of the clinical and immunopathological characteristics of 34 patients with MMP that were treated at the Blistering Disease Clinic at the Dermatology Department at the Ramos Mejia Hospital between January 1997 and March 2021.Materials and methods: though the medical histories and the photographic registries, we evaluated the following variables: prevalence of MMP within the patients that came to consult at the Blistering Disease Clinic at the Dermatology Department, sex, age, personal history, the affected mucous, evolution time until the diagnosis, direct immunofluorescence findings, clinical follow-ups and treatments.Results: we studied the clinical evolution of 34 patients diagnosed with MMP at our institution (5.3% from the total of patients at the Blistering Disease Clinic).The most affected gender was female and the average age at diagnosis was 64 years. 70.6% presented comorbidities such as hypertension and hypothyroidism. Most of our patients referred an emotional triggering event. The most affected membrane mucous was the ocular one and the oral was the second one. The delay in diagnosis was 4 years and 11 months. Linear deposits of IgG was the most frequent result in the direct immunofluorescence. 17.6% did not continue clinical follow-up. Mycophenolate mofetil was the most used drug with a good therapeutic response. Conclusions: MMP is a rare autoimmune disease that affects mucous membrane and occasionally the skin. In this study, the ocular involvement was the most frequent one, differing with the international reports. The early interdisciplinary diagnosis is essential to avoid irreversible sequelae.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Penfigoide Mucomembranoso Benigno , Penfigoide Bolhoso/diagnóstico , Mucosa Esofágica , Mucosa BucalRESUMO
Se actualiza el diagnóstico de la urticaria crónica (UC) y los conceptos, definiciones y sugerencias basados en la evidencia para su tratamiento. La urticaria ocurre en al menos 20% de la población en algún momento de la vida. Su etiología difiere en la forma aguda (menos de 6 semanas), y en la crónica. No es posible pronosticar si las formas agudas evolucionarán a UC, ya que todas son agudas al comienzo. La UC ocurre como espontánea (UCE) o inducible (UCI). El diagnóstico es sencillo, pero incluye un minucioso estudio para descartar diagnósticos diferenciales; para UCI son útiles las pruebas de provocación en la caracterización y manejo. Los estudios complementarios se deben limitar y orientar según sospecha clínica. El tratamiento se divide en tres enfoques: evitación, eliminación o tratamiento del estímulo desencadenante o de la causa, y tratamiento farmacológico. Recientemente éste se modificó, con empleo de antihistamínicos de segunda generación como primera línea y aumento de dosis de antihistamínicos H1 no sedantes, hasta 4 veces, como segunda línea. Los antihistamínicos son fundamentales para tratar la UC; sin embargo, un 40% de los pacientes no logra un buen control pese al aumento de dosis y requiere otro medicamento adicional. La evidencia más reciente considera que un grupo de fármacos puede utilizarse como tercera línea en estos casos, para mejorar la calidad de vida y limitar la toxicidad por el uso frecuente o crónico de esteroides sistémicos. Se recomiendan para esta tercera línea solo 3 fármacos: omalizumab, ciclosporina A o antileucotrienos.
This interdisciplinary paper summarizes the news in the diagnosis and treatment of chronic urticaria (CU), and provides concepts, definitions and evidence-based suggestions for its management. Urticaria occurs in at least 20% of the population at some point in their lives. Acute urticaria (less than 6 weeks' duration), differs from CU in its etiology, but the onset of this disease is always acute. CU may occur as spontaneous (SCU) or induced (ICU). The diagnosis is simple, although a careful evaluation is necessary for differential diagnosis. ICU´s diagnosis is mainly clinical, even if provocation tests can be useful. Supplementary studies should be limited and based on the clinical suspicion. Treatment may be divided into three approaches: avoidance, elimination or treatment of the cause, and pharmacological treatment. Recently treatment has been modified with the use of second-generation antihistamines as first-line and increased doses of nonsedating H1 antihistamines, up to 4 times, as second line. Antihistamines are essential to treat CU; however, 40% of patients do not achieve good control despite increased doses and require additional treatment. The most recent evidence indicates a group of drugs to be used as third line in these cases, to improve quality of life and to limit toxicity from frequent or chronic use of systemic steroids. Only 3 drugs are recommended as third line: omalizumab, cyclosporin A or anti-leukotrienes.
Assuntos
Humanos , Antialérgicos/uso terapêutico , Antagonistas dos Receptores Histamínicos/uso terapêutico , Urticária/diagnóstico , Urticária/tratamento farmacológico , Urticária/etiologia , Algoritmos , Argentina , Angioedema/tratamento farmacológico , Angioedema/patologia , Anticorpos Anti-Idiotípicos/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Doenças Autoimunes/complicações , Doença Crônica , Ensaios Clínicos como Assunto , Ciclosporina/uso terapêutico , Diagnóstico Diferencial , Medicina Baseada em Evidências/economia , Imunoglobulina E/metabolismo , Antagonistas de Leucotrienos/uso terapêutico , Omalizumab , Qualidade de Vida , Urticária/classificação , Urticária/complicações , Urticária/fisiopatologiaRESUMO
This interdisciplinary paper summarizes the news in the diagnosis and treatment of chronic urticaria (CU), and provides concepts, definitions and evidence-based suggestions for its management. Urticaria occurs in at least 20
of the population at some point in their lives. Acute urticaria (less than 6 weeks duration), differs from CU in its etiology, but the onset of this disease is always acute. CU may occur as spontaneous (SCU) or induced (ICU). The diagnosis is simple, although a careful evaluation is necessary for differential diagnosis. ICUs diagnosis is mainly clinical, even if provocation tests can be useful. Supplementary studies should be limited and based on the clinical suspicion. Treatment may be divided into three approaches: avoidance, elimination or treatment of the cause, and pharmacological treatment. Recently treatment has been modified with the use of second-generation antihistamines as first-line and increased doses of nonsedating H1 antihistamines, up to 4 times, as second line. Antihistamines are essential to treat CU; however, 40
of patients do not achieve good control despite increased doses and require additional treatment. The most recent evidence indicates a group of drugs to be used as third line in these cases, to improve quality of life and to limit toxicity from frequent or chronic use of systemic steroids. Only 3 drugs are recommended as third line: omalizumab, cyclosporin A or anti-leukotrienes.
Assuntos
Humanos , Urticária/diagnóstico , Urticária/etiologia , Urticária/tratamento farmacológico , Antialérgicos/uso terapêutico , Antagonistas dos Receptores Histamínicos/uso terapêutico , Argentina , Qualidade de Vida , Urticária/fisiopatologia , Algoritmos , Doença Crônica , Ensaios Clínicos como Assunto , Diagnóstico Diferencial , Omalizumab , Angioedema/tratamento farmacológicoRESUMO
BACKGROUND & AIMS: Dermatitis herpetiformis (DH) is characterized by variable degrees of enteropathy and increased intestinal permeability. Zonulin, a regulator of tight junctions, seems to play a key role in the altered intestinal permeability that characterizes the early phase of celiac disease. Our aim was to assess both intestinal permeability and serum zonulin levels in a group of patients with DH having variable grades of enteropathy. METHODS: We studied 18 DH patients diagnosed on the basis of characteristic immunoglobulin (Ig)A granular deposits in the dermal papillae of noninvolved skin. Results were compared with those of classic celiac patients, patients with linear IgA dermatosis, and healthy controls. RESULTS: According to Marsh's classification, 5 patients had no evidence of enteropathy (type 0), 4 patients had type II, 2 patients had type IIIb damage, and 7 patients had a more severe lesion (type IIIc). Intestinal permeability (lactulose/mannitol ratio [lac/man]) was abnormal in all patients with DH. Patients with more severe enteropathy had significantly greater permeability ( P < .05). The serum zonulin concentration (enzyme-linked immunosorbent assay) for patients with DH was 2.1 +/- .3 ng/mg with 14 of 16 (87.5%) patients having abnormally increased values. In contrast, patients with linear IgA dermatosis had normal histology, normal intestinal permeability, and negative celiac serology. CONCLUSIONS: Increased intestinal permeability and zonulin up-regulation are common and concomitant findings among patients with DH, likely involved in pathogenesis. Increased permeability can be observed even in patients with no evidence of histologic damage in biopsy specimens. Patients with linear IgA dermatosis appear to be a distinct population with no evidence of gluten sensitivity.
Assuntos
Doença Celíaca/diagnóstico , Doença Celíaca/epidemiologia , Toxina da Cólera/biossíntese , Dermatite Herpetiforme/diagnóstico , Dermatite Herpetiforme/epidemiologia , Adolescente , Adulto , Distribuição por Idade , Idoso , Biomarcadores/análise , Estudos de Casos e Controles , Toxina da Cólera/sangue , Comorbidade , Feminino , Haptoglobinas , Humanos , Incidência , Absorção Intestinal/fisiologia , Masculino , Pessoa de Meia-Idade , Permeabilidade , Probabilidade , Prognóstico , Precursores de Proteínas , Valores de Referência , Sensibilidade e Especificidade , Índice de Gravidade de Doença , Distribuição por SexoRESUMO
Background: Dermatitis herpetiformis (DH), a wellestablished gluten-sensitive skin disorder presenting variable degrees of enteropathy, constitutes a very useful model in order to assess the utility of the celiac disease (CD)-related serology in patients with mild intestinal damage. Objective: Our aim was to explore comparatively the performance of a panel of CD-related serologic tests in patients with DH. Methods: We assessed a series of 18 consecutive patients with skin biopsy proven DH presenting the overall spectrum of intestinal damage ranging from normal mucosa (n=6) to total villous atrophy (TVA) (n=6) through partial villous atrophy (PVA) (n=6). Sera were obtained from all patients while consuming a gluten containing diet. Serologic tests were antiendomysial, anti-tissue transglutaminase and antigliadin antibodies, and newly developed tests detecting both antibody isotypes (IgA and IgG) against deamidated synthetic gliadin-derivedpeptides (a-GDP). Results: Serologic tests had a variable behaviour depending on the degree of enteropathy. While the majority of tests detected patients with TVA, only 50% of those with normal histology had positive assays. Patients with PVA had discordant results. Classical CD-specific tests were positive in only some patients with mild damage while all of them were identified by a single assay detecting both isotypes of a-GDP. Conclusion: The detection of a-GDP antibodies was the most reliable tool in order to identify gluten sensitivity in DH patients presenting a wide range of intestinal damage. Further studies should explore if these findings can be extrapolated to patients with CDhaving mild enteropathy.
Introducción: la dermatitis herpetiformis (DH), una lesión dermatológica consecuencia de sensibilidad al gluten y asociada a grados variables de enteropatía, constituye un modelo muy útil con el objeto de evaluar la eficacia de la serología de la enfermedad celíaca(EC) en pacientes con daño intestinal leve. Objetivo: explorar comparativamente la utilidad de una serie de anticuerpos empleados en EC en pacientes con DH. Métodos: analizamos una serie de 18 pacientes consecutivos con diagnóstico de DH por biopsia de piel que presentaban el más amplio espectro de daño intestinal variando desde una mucosa normal (n=6) a la atrofia vellosa total (AVT) (n=6) y pasando por atrofia vellosaparcial (AVP) (n=6). Se obtuvo plasma de todos los pacientes mientras consumían gluten. Las pruebas serológicas empleadas fueron anticuerpos antiendomisio, anti-transglutaminasa y atigliadina, y unas pruebas recientemente desarrolladas que detectan anticuerpos IgA e IgG dirigidos contra péptidos sintéticos deamidados derivados de la gliadina (a-GDP). Resultados: las diferentes pruebas tuvieron un comportamiento variable dependiendo del grado de lesión intestinal. Mientras que la mayoría de las pruebas detectaron a todos los pacientes con AVT, sólo el 50% de aquellos con histologíanormal tuvieron resultados positivos. Los pacientes con AVP tuvieron resultados discordantes. Así las pruebas clásicas fueron positivas en sólo algunos pacientescon daño leve, mientras que todos ellos fueron positivos a una prueba para detectar ambos isotipos del a-GDP. Conclusión: la determinación de anticuerpos a-GDP fue la herramienta más confiable con el objeto de identificar serológicamente la sensibilidad al gluten en pacientes con DH que presentan variables grados de daño intestinal. Otros estudios deberían explorar si estos hallazgos podrían ser extrapolados a pacientes conEC con enteropatía de grado leve.
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso de 80 Anos ou mais , Autoanticorpos/sangue , Doença Celíaca/diagnóstico , Dermatite Herpetiforme/diagnóstico , Atrofia , Biomarcadores/sangue , Biópsia , Doença Celíaca/imunologia , Doença Celíaca/patologia , Dermatite Herpetiforme/imunologia , Dermatite Herpetiforme/patologia , Gliadina/imunologia , Imunoglobulina A/sangue , Imunoglobulina G/sangue , Valor Preditivo dos Testes , Estudos Prospectivos , Índice de Gravidade de Doença , Transglutaminases/imunologiaRESUMO
La dermatitis herpetiformis es una dermatosis autoinmune que ocurre como resultado de la ingesta de gluten en individuos genéticamente predispuestos. Actualmente es considerada como la manifestación cutánea de la enfermedad celíaca. Cursa con brotes de lesiones pápulo-vesico-ampollares extremadamente pruriginosas, localizadas preferentemente en cuero cabelludo, superficies de extensión de codos y rodillas, glúteos y dorso y tiene evolución crónica. Ocurre más comúnmente en adultos jóvenes, pero también afecta a niños, en los que puede tener presentaciones clínicas atípicas. Todos los pacientes con dermatitis herpetiformis presentan algún grado de compromiso intestinal. El diagnóstico de certeza se realiza por el hallazgo patognomónico de depósitos granulares de inmunoglobulina A en la unión dermoepidérmica con acentuación en las cimas de las papilas dérmicas, en piel sana perilesional. El tratamiento sintomático se logra con el uso de dapsona, pero el único definitivo es la adherencia a largo plazo a una dieta libre de gluten
Dermatitis herpetiformis is an autoimmune dermatosis that results from gluten ingestion in genetically predisposed patients. It is currently considered the cutaneous manifestation of celiac disease. It presents with crops of extremely pruriginous papular and vesicobullous lesions located mainly at scalp, extensor surfaces of elbows and knees, buttocks and back; and has a chronic course. Although more frequent in young adults, it also affects childs, in whom may present with atypical skin manifestations. All patients with dermatitis herpetiformis have some degree of intestinal compromise. Definitive diagnosis is based on the pathognomonic finding of a granular IgA deposition at the dermoepidermal junction with stippling in the dermal papillae, in normal appearing perilesional skin. Symptomatic treatment is achieved with dapsone use, but definitive one is based on lifelong adherence to a gluten-free diet
Assuntos
Humanos , Adolescente , Adulto , Criança , Doença Celíaca , Dermatite Herpetiforme , Glutens/efeitos adversos , Glutens/toxicidadeRESUMO
Se presenta el caso de una niña de un año y medio con diagnóstico clínico, inmunopatológico y técnica de salt-split de epidermólisis ampollar adquirida. Se efectuó una revisión del tema de los casos pediátricos presentados hasta la fecha; nueve en total