RESUMO
Tuberous sclerosis complex manifests predominantly as a neurocutaneous disorder Lung involvement was considered rare. Lymphangioleiomyomatosis which occurs mainly in women of childbearing age is the major pulmonary disorder seen in tuberous sclerosis. Multifocal micronodular pneumocyte hyperplasia has also been described in tuberous sclerosis. The case of a 51-year old female diagnosed with tuberous sclerosis is described after she presented with progressive shortness of breath and was found to have interstitial lung disease. Tuberous sclerosis should be considered as a differential in patients with interstitial lung disease especially in association with cutaneous lesions.
Assuntos
Hiperplasia/patologia , Pneumopatias/etiologia , Pulmão/patologia , Esclerose Tuberosa/patologia , Dor no Peito/diagnóstico , Progressão da Doença , Dispneia/diagnóstico , Feminino , Humanos , Hiperplasia/diagnóstico , Pneumopatias/diagnóstico , Pneumopatias/patologia , Pessoa de Meia-Idade , Dermatopatias/etiologia , Esclerose Tuberosa/complicações , Esclerose Tuberosa/diagnósticoRESUMO
Tuberous sclerosis complex manifests predominantly as a neurocutaneous disorder Lung involvement was considered rare. Lymphangioleiomyomatosis which occurs mainly in women of childbearing age is the major pulmonary disorder seen in tuberous sclerosis. Multifocal micronodular pneumocyte hyperplasia has also been described in tuberous sclerosis. The case of a 51-year old female diagnosed with tuberous sclerosis is described after she presented with progressive shortness of breath and was found to have interstitial lung disease. Tuberous sclerosis should be considered as a differential in patients with interstitial lung disease especially in association with cutaneous lesions.
El complejo de esclerosis tuberosa se manifiesta predominantemente como un desorden neurocutáneo. El compromiso pulmonar era considerado raro. La linfangioleiomiomatosis que se presenta principalmente en mujeres en estado de gestación, es el principal trastorno observado en la esclerosis tuberosa. La hiperplasia micronodular pneumocítica ha sido también descrita en la esclerosis tuberosa. Se describe el caso de una mujer de 51 años a quien se le diagnosticó esclerosis tuberosa, luego de que se presentara con disnea y se hallara que padecía la enfermedad intersticial del pulmón. La esclerosis tuberosa deber ser considerada como un diagnóstico diferencial en pacientes con la enfermedad intersticial del pulmón, especialmente cuando se encuentra asociada con lesiones cutáneas.