RESUMEN
BACKGROUND: Linear IgA bullous dermatosis (LAD) of childhood is a rare acquired subepidermal blistering disease of young children. Most of the studies were reported from the USA and European countries. METHOD: Twelve cases of Thai patients diagnosed as LAD of childhood were analyzed concerning clinical, histopathological, immunopathological findings including treatment responses and courses compared with those of Caucasians. RESULT: The mean age of onset was 5.1 years. The areas of common involvement were the perioral region, lower abdomen, perineum, buttock, inner thighs and extremities. Histopathology in half of the cases showed features of dermatitis herpetiformis or bullous pemphigoid. All patients had positive linear IgA band at the basement membrane zone (BMZ) by direct immunofluorescence. Only one patient had positive circulating anti BMZ antibody at the titer of 1:10. Most patients responded well to dapsone. The mean duration before remission was 1.9 years. CONCLUSION: Our study in Thai patients with LAD of childhood produced data similar to previous studies carried out in the Caucasian nations.
Asunto(s)
Adolescente , Antiinflamatorios no Esteroideos/administración & dosificación , Niño , Preescolar , Dapsona/administración & dosificación , Estudios de Evaluación como Asunto , Femenino , Técnica del Anticuerpo Fluorescente Indirecta , Humanos , Inmunoglobulina A/análisis , Masculino , Pronóstico , Índice de Severidad de la Enfermedad , Enfermedades Cutáneas Vesiculoampollosas/tratamiento farmacológico , TailandiaAsunto(s)
Adulto , Femenino , Humanos , Lupus Eritematoso Discoide , Masculino , Persona de Mediana Edad , PénfigoRESUMEN
Correct clinical diagnosis in cases of chronic, relapsing, non-hereditary, blistering diseases in childhood could not be made without the aid of histopathology and immunofluorescence, since the morphology and the distribution of the lesions of bullous pemphigoid (BP), linear IgA bullous dermatosis (LAD) and dermatitis herpetiformis (DH) may be similar. Histopathology was helpful in about half of the cases. The results of immunopathology were very useful for the final diagnosis. Of twenty-one cases of juvenile blistering diseases, two cases which showed IgG on direct test with circulating antibodies were BP; three cases with deposition of IgG but without circulating antibodies were probably BP; three cases were either BP or LAD (IgG and IgA on direct test without circulating antibodies); nine cases were definite LAD (linear IgA only); one case which showed granular IgA in the dermal papillae and linear IgA was DH; and the last three cases were probably LAD and BP with non-immunoreactant deposits. Regardless of the diagnosis, dapsone and co-trimoxazole controlled eight cases and could be discontinued without relapse, while the other six cases were maintained on dapsone therapy alone. Prednisolone, when added in cases of poor response to either dapsone or co-trimoxazole, caused remission and was discontinued in three out of the five cases. Two cases were lost to follow up.