RESUMEN
BACKGROUND: Behçet's disease is a chronic, relapsing disease, about which information on its clinical course in juveniles is only available from small groups of patients. MATERIALS AND METHODS: Patients suffering from Behçet's disease who had their first lesion at or before the age of 16 were evaluated in terms of: age at onset, mucocutaneous signs, and findings related to systemic involvement. Ninety-five patients, evaluated as having juvenile Behçet's disease (JBD), were detected among 1784 Turkish Behçet's patients. The mean age of these 95 patients (51 boys or men, 44 girls or women) who had JBD was 26.8 +/- 7.1 years. RESULTS: The difference between sexes in terms of age at onset, development period of second lesions, and systemic involvement was not found to be significant in JBD. Patients were divided into two groups, one showing severe disease (N = 27) and the other mild disease (N = 68). There was no significant difference between the two groups with respect to age, age at onset, and sex distribution. The interval between the development of the first and second lesions was shorter in the patient group with severe disease (P < 0.001) and the development of second lesion was most frequently seen in the first 5 years (P < 0.05). Systemic involvement developed also in a shorter time in the group with the severe disease (P < 0.01) and was most frequently encountered during the first 5 years (P < 0.05). Conversely, patients with the mild disease developed systemic involvement more frequently after 6 years or later. CONCLUSIONS: Severe Behçet's disease in children and juveniles shows no age or sex predilection, but leads to an earlier recurrence and more severe systemic signs than the mild form.
Asunto(s)
Síndrome de Behçet/patología , Adolescente , Adulto , Factores de Edad , Edad de Inicio , Análisis de Varianza , Síndrome de Behçet/epidemiología , Niño , Femenino , Estudios de Seguimiento , Humanos , Masculino , Membrana Mucosa/patología , Recurrencia , Distribución por Sexo , Factores Sexuales , Úlcera Cutánea/patología , Estomatitis Aftosa/patología , Factores de Tiempo , Turquía/epidemiologíaRESUMEN
BACKGROUND: There is no definite treatment for Behçet's disease. New drugs are being evaluated in cases unresponsive to conventional treatment modalities. OBJECTIVE: In this study we investigated the efficacy of interferon alfa-2a on skin lesions in 18 Behçet's disease patients who had predominantly mucocutaneous involvement. METHODS: Eighteen patients with Behçet's disease were treated with interferon alfa-2a at 3 million IU/day in the first week (three times a week), 6 million IU/day in the second week (three times a week), 9 million IU/day in the third week and thereafter (three times a week). Interferon alfa-2a was administered subcutaneously for a total of 12 weeks. RESULTS: At the end of the treatment the efficacy of the drug was found to be good in 9 patients and very good in 7 patients. Interferon alfa-2a is particularly effective in skin manifestations such as genital ulceration and erythema-nodosum-like lesions. It was also found to be effective in systemic manifestations such as fever, diarrhea and eye involvement. CONCLUSION: Our results indicate that interferon alfa-2a is a promising drug in the treatment of Behçet's disease.
Asunto(s)
Síndrome de Behçet/terapia , Interferón-alfa/uso terapéutico , Adulto , Femenino , Estudios de Seguimiento , Humanos , Inyecciones Subcutáneas , Interferón alfa-2 , Interferón-alfa/administración & dosificación , Masculino , Persona de Mediana Edad , Proteínas Recombinantes , Resultado del TratamientoRESUMEN
The HLA-B5 phenotype was investigated in 235 patients with Behçet's disease and in 100 healthy persons. HLA-B5 was more frequent in the patients (77 vs. 30% p < 0.001, relative risk = 7.8). The frequency of HLA-B5 was higher in patients with genital ulceration than without (82.3 vs. 63%, p < 0.01). Patients with thrombophlebitis showed a less frequent positivity of HLA-B5 antigen than the patients without thrombophlebitis (50 vs. 79.2%, p < 0.02). These results suggest that HLA-B5-related genes not only affect the development of Behçet's disease but also the occurrence of its clinical manifestations.
Asunto(s)
Síndrome de Behçet/inmunología , Antígenos HLA-B/análisis , Adolescente , Adulto , Artritis/inmunología , Artritis/patología , Síndrome de Behçet/genética , Síndrome de Behçet/patología , Eritema Nudoso/inmunología , Eritema Nudoso/patología , Oftalmopatías/inmunología , Oftalmopatías/patología , Femenino , Foliculitis/inmunología , Foliculitis/patología , Enfermedades de los Genitales Femeninos/inmunología , Enfermedades de los Genitales Femeninos/patología , Enfermedades de los Genitales Masculinos/inmunología , Enfermedades de los Genitales Masculinos/patología , Antígenos HLA-B/genética , Humanos , Masculino , Persona de Mediana Edad , Fenotipo , Factores de Riesgo , Estomatitis Aftosa/inmunología , Estomatitis Aftosa/patología , Tromboflebitis/inmunología , Tromboflebitis/patología , Úlcera/inmunología , Úlcera/patologíaRESUMEN
A new skin lesion was encountered in 29 of 970 Behçet's patients. The lesions resembled oral aphthae clinically, were recurrent and left a scar tissue like genital ulcers but were located extragenitally. Skin biopsies could be done in only 4 cases and they all showed vasculitis.
Asunto(s)
Síndrome de Behçet/patología , Adulto , Femenino , Estudios de Seguimiento , Humanos , Masculino , Recurrencia , Úlcera Cutánea/patologíaRESUMEN
A positive pathergy test in patients with Behçet's disease has been accepted as a diagnostic criterion by many authors, but in recent years it has been claimed that the test has a decreased positivity. We have examined the test in 92 proven cases of Behçet's disease, using 20G and 26G disposable needles and evaluated them after 48 h. Maximum positivity was found to be 65% when we used needles of size 0.9 mm (20G), but the reactivity was considerably less when 0.3 mm diameter (26G) needles were used. The disposable needles used nowadays are less traumatic to initiate the reaction than were the non-disposable ones used in the pre-AIDS era.
Asunto(s)
Síndrome de Behçet/diagnóstico , Pruebas Cutáneas/instrumentación , Adulto , Equipos Desechables , Femenino , Humanos , Masculino , Agujas , Valor Predictivo de las PruebasRESUMEN
In the last 12 years, we treated 427 Turkish patients suffering from Behçet's disease. We report on the frequency of symptoms as well as the modes of therapy in Turkey.