RESUMO
Sarcoidosis is a multisystemic granulomatous disorder of unknown etiology, most commonly affecting young adults and presenting most frequently with bilateral hilar lymphadenopathy, pulmonary infiltration, and skin or eye lesions. Sarcoidosis in children under the age of 4, preschool sarcoidosis, is rare and has very different clinical mainfestations characterized by the typical triad of skin, eye and joint involvement without lung disease. Diagnosis is established when compatible clinical findings are supported by histological evidence of widespread noncaseating epithelioid-cell granulomas in more than one organ or a positive Kveim-Siltzbach skin test. This report describes a 2-year-old Thai girl with typical clinical manifestations of preschool sarcoidosis. The histopathological studies of tissue specimens and the laboratory investigations support the diagnosis of sarcoidosis. The patient responded to systemic corticosteroid therapy.
Assuntos
Corticosteroides/uso terapêutico , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Fígado/patologia , Sarcoidose/diagnóstico , Pele/patologiaRESUMO
This is a report of a case, 7 1/2 year-old-boy having chronic febrile and recurrent crops of painful subcutaneous nodules on lower extremities, which had previously been diagnosed as Weber-Christian disease, which progressed to have cytophagic histiocytic activity in the skin, bone marrow with abnormal liver function and hemorrhagic diathesis. He was subsequently treated with corticosteroid without good response. After he was diagnosed as having cytophagic histiocytic panniculitis, cyclosporin A was administered intravenously in an initial dosage of 1 mg/kg/day and in oral maintenance dose of 10 mg/kg/day with a successful response and the patient completely recovered within 6 months with mild hypertension as an adverse effect.