RESUMO
BACKGROUND: The Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires proved to be reliable tools that measure the disease and treatment burden. OBJECTIVES: We aimed to assess the ABQOL and TABQOL in the Arabic population. METHODS: The English questionnaires were translated into the Arabic language by a certified translation agency. Eighty autoimmune bullous disease (AIBD) patients were included in this study. Patients were asked to answer 2 questionnaires. After 1 week the same patients were asked to answer the same questionnaires again. RESULTS: The age of the patients ranged from 19 to 81 years (mean=46), 19 males, 61 females. The ABQOL ranged from 0-37 (mean=16.4±9.2). The TABQOL ranged from 2-43 (mean=21.5±9.4). Test-retest reliability was acceptable, Cronbach's alpha was 0.76 for ABQOL and 0.74 for TABQOL. There was no significant correlation between the age of the patients and ABQOL, r =-0.2, p value was 0.183. There was a significant negative correlation between the age of the patients and the TABQOL, r=-0.2, p value was 0.039. There was a significant negative correlation between the education of the patients and the TABQOL, r=-0.3, p value was 0.007. STUDY LIMITATIONS: Small sample size of some AIBDs and patients with severe disease. CONCLUSION: Objective and valuable measurements such as ABQOL and TABQOL are now available to help physicians understand their patient's distress and should be used in every patient with AIBD. Younger and less educated patients appear to have more effects on their QOL from the treatments.
Assuntos
Doenças Autoimunes/fisiopatologia , Doenças Autoimunes/terapia , Qualidade de Vida , Dermatopatias Vesiculobolhosas/fisiopatologia , Dermatopatias Vesiculobolhosas/terapia , Inquéritos e Questionários/normas , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos Transversais , Egito , Feminino , Humanos , Idioma , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Reprodutibilidade dos Testes , Índice de Gravidade de Doença , Dermatopatias Vesiculobolhosas/imunologia , Fatores de Tempo , Resultado do Tratamento , Tunísia , Adulto JovemRESUMO
Abstract: Background: The Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires proved to be reliable tools that measure the disease and treatment burden. Objectives: We aimed to assess the ABQOL and TABQOL in the Arabic population. Methods: The English questionnaires were translated into the Arabic language by a certified translation agency. Eighty autoimmune bullous disease (AIBD) patients were included in this study. Patients were asked to answer 2 questionnaires. After 1 week the same patients were asked to answer the same questionnaires again. Results: The age of the patients ranged from 19 to 81 years (mean=46), 19 males, 61 females. The ABQOL ranged from 0-37 (mean=16.4±9.2). The TABQOL ranged from 2-43 (mean=21.5±9.4). Test-retest reliability was acceptable, Cronbach's alpha was 0.76 for ABQOL and 0.74 for TABQOL. There was no significant correlation between the age of the patients and ABQOL, r =-0.2, p value was 0.183. There was a significant negative correlation between the age of the patients and the TABQOL, r=-0.2, p value was 0.039. There was a significant negative correlation between the education of the patients and the TABQOL, r=-0.3, p value was 0.007. Study limitations: Small sample size of some AIBDs and patients with severe disease. Conclusion: Objective and valuable measurements such as ABQOL and TABQOL are now available to help physicians understand their patient's distress and should be used in every patient with AIBD. Younger and less educated patients appear to have more effects on their QOL from the treatments.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Adulto Jovem , Qualidade de Vida , Doenças Autoimunes/fisiopatologia , Doenças Autoimunes/terapia , Inquéritos e Questionários/normas , Dermatopatias Vesiculobolhosas/fisiopatologia , Dermatopatias Vesiculobolhosas/terapia , Fatores de Tempo , Tunísia , Índice de Gravidade de Doença , Estudos Transversais , Análise Multivariada , Reprodutibilidade dos Testes , Dermatopatias Vesiculobolhosas/imunologia , Resultado do Tratamento , Egito , IdiomaAssuntos
Doenças Autoimunes/diagnóstico , Prurido/diagnóstico , Dermatopatias Vesiculobolhosas/diagnóstico , Pele/patologia , Anti-Infecciosos/uso terapêutico , Doenças Autoimunes/fisiopatologia , Dapsona/uso terapêutico , Eritromicina/uso terapêutico , Feminino , Humanos , Imunoglobulina A/metabolismo , Lactente , Microscopia de Fluorescência/métodos , Prurido/terapia , Dermatopatias Vesiculobolhosas/fisiopatologiaRESUMO
BACKGROUND: Papular urticaria caused by flea bite presents clinical symptoms of a hypersensitivity reaction accompanied by skin lesions. However, the pattern of recognition by different antibody isotypes during the progression of the disease is unknown. This study evaluated variations in immunoglobulin E and immunoglobulin G subclass antibody responses to flea antigens during the progression of papular urticaria caused by flea bite METHODS: Twenty-five patients clinically diagnosed with papular urticaria due to flea bite were included. Ten healthy children were included as controls. Recognition of antigens from complete flea body extract by patients and healthy controls was determined using immunoblot assays. RESULTS: The results revealed that patients with 2-5 years of papular urticaria evidenced more IgE bands than those with shorter or longer durations of symptoms. In contrast, healthy children showed a predominance of immunoglobulin G1 and immunoglobulin G3. The majority of the recognised antigens were low molecular weight proteins (<90 kDa). Proteins with molecular weights between 16-20, 21-25, and 31-35 kDa showed different patterns of recognition between patients and healthy children. CONCLUSION: The predominant specific antibody isotypes vary according to the time elapsed since the onset of symptoms in papular urticaria caused by flea bite.
Assuntos
Mordeduras e Picadas/imunologia , Epitopos/imunologia , Imunoglobulina E/imunologia , Imunoglobulina G/imunologia , Adolescente , Animais , Mordeduras e Picadas/complicações , Mordeduras e Picadas/diagnóstico , Mordeduras e Picadas/fisiopatologia , Criança , Pré-Escolar , Progressão da Doença , Epitopos/metabolismo , Feminino , Humanos , Lactente , Masculino , Sifonápteros , Dermatopatias Vesiculobolhosas/diagnóstico , Dermatopatias Vesiculobolhosas/etiologia , Dermatopatias Vesiculobolhosas/imunologia , Dermatopatias Vesiculobolhosas/fisiopatologia , Urticária/diagnóstico , Urticária/etiologia , Urticária/imunologia , Urticária/fisiopatologiaRESUMO
Se realiza una revisión bibliográfica de casos de poiquilodermia Acroqueratótica Hereditaria o enfermedad de Weary (EW). Hemos concluido que dicha denominación pareciera ser usada en cuatro diferentes cuadros dermatológicos: 1) Enfermedad de Weary Acroqueratótica Hereditaria con herencia familiar autosómica dominante (EW-d); 2) Enfermedad de Weary, con herencia familiar autosómica recesiva y algunos rasgos clínicos de una EW-d asociada a manifestaciones de fotosensibilidad de un síndrome de Kindler; 3) Síndrome de Kindler-Weary (SK-W) con lesiones clínicas de un síndrome de Kindler (poliquilodermia con ampollas traumáticas y atrofia cutánea progresiva) e histopatológicas con manifestaciones liquenoides y presencia de "cuerpos citoides" de una EW-d y 4) Síndrome de Weary-Kindler con manifestaciones de una Poiquilodermia Esclerosante Hereditaria de Weary (HSP), asociada también a manifestaciones ampollares de un SK-r (WE-KS-r). Nosotros relatamos un nuevo caso clínico e histopatológico de una EW-d con presencia de "cuerpos citoides", como expresión de una reacción inmunológica mediada por células, demostrable por inmunohistoquímica. En el diagnóstico diferencial se destaca la distinta patogenia del SK que presenta una malformación displásica genética de la membrana basal electrónica (láminas lúcida y densa) y del tejido conectivo papilar dérmico con desaparición de las fibras elásticas. Nuestra revisión bibliográfica demostró que la EW-d y el SK-r representan dos "formas polares" patogénicas: una predominantemente inmunológica y la segunda malformativa displásica genética, en un amplio espectro de Poiquilodermias Ampollares Hereditarias (HAP), vinculadas con otras cuatro "formas subpolares": EW-r, SK-r, WE-KS-r y SK-d. Estas conclusiones están basadas en el análisis patogénico, clínico, histopatológico, inmunohistoquímico y ultraestructural de componentes del HAP
Assuntos
Humanos , Pré-Escolar , Feminino , Dermatopatias Genéticas/diagnóstico , Dermatopatias Vesiculobolhosas/diagnóstico , Síndrome de Rothmund-Thomson/diagnóstico , Dermatopatias Genéticas/fisiopatologia , Dermatopatias Genéticas/patologia , Dermatopatias Vesiculobolhosas/fisiopatologia , Dermatopatias Vesiculobolhosas/patologia , Síndrome de Rothmund-Thomson/classificação , Síndrome de Rothmund-Thomson/genéticaRESUMO
Se realiza una revisión bibliográfica de casos de poiquilodermia Acroqueratótica Hereditaria o enfermedad de Weary (EW). Hemos concluido que dicha denominación pareciera ser usada en cuatro diferentes cuadros dermatológicos: 1) Enfermedad de Weary Acroqueratótica Hereditaria con herencia familiar autosómica dominante (EW-d); 2) Enfermedad de Weary, con herencia familiar autosómica recesiva y algunos rasgos clínicos de una EW-d asociada a manifestaciones de fotosensibilidad de un síndrome de Kindler; 3) Síndrome de Kindler-Weary (SK-W) con lesiones clínicas de un síndrome de Kindler (poliquilodermia con ampollas traumáticas y atrofia cutánea progresiva) e histopatológicas con manifestaciones liquenoides y presencia de "cuerpos citoides" de una EW-d y 4) Síndrome de Weary-Kindler con manifestaciones de una Poiquilodermia Esclerosante Hereditaria de Weary (HSP), asociada también a manifestaciones ampollares de un SK-r (WE-KS-r). Nosotros relatamos un nuevo caso clínico e histopatológico de una EW-d con presencia de "cuerpos citoides", como expresión de una reacción inmunológica mediada por células, demostrable por inmunohistoquímica. En el diagnóstico diferencial se destaca la distinta patogenia del SK que presenta una malformación displásica genética de la membrana basal electrónica (láminas lúcida y densa) y del tejido conectivo papilar dérmico con desaparición de las fibras elásticas. Nuestra revisión bibliográfica demostró que la EW-d y el SK-r representan dos "formas polares" patogénicas: una predominantemente inmunológica y la segunda malformativa displásica genética, en un amplio espectro de Poiquilodermias Ampollares Hereditarias (HAP), vinculadas con otras cuatro "formas subpolares": EW-r, SK-r, WE-KS-r y SK-d. Estas conclusiones están basadas en el análisis patogénico, clínico, histopatológico, inmunohistoquímico y ultraestructural de componentes del HAP (AU)