Steroid-Responsive Rosai-Dorfman Disease
Autor: | Salim M. Farid, Atilio Baez-Giangreco, John I. Antonius |
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Rok vydání: | 1996 |
Předmět: |
medicine.medical_specialty
Prednisolone Administration Oral Diagnosis Differential Rhabdomyosarcoma Biopsy medicine Humans Rosai–Dorfman disease medicine.diagnostic_test Vascular disease business.industry Infant Newborn Infant Sinus Histiocytosis with Massive Lymphadenopathy Hematology Inguinal lymphadenopathy Angiomatosis medicine.disease Dermatology Surgery Histiocytosis Oncology Child Preschool Pediatrics Perinatology and Child Health Female Histiocytosis Sinus medicine.symptom business medicine.drug |
Zdroj: | Pediatric Hematology and Oncology. 13:563-570 |
ISSN: | 1521-0669 0888-0018 |
DOI: | 10.3109/08880019609030873 |
Popis: | Nodular skin lesions on the lateral aspects of the legs of a female child were first noticed at the age of 6 days. A biopsy of the lesions was done at the age of 6 months when the child had also developed cervical and inguinal lymphadenopathy and angiomatous lesions on the face. The diagnosis of Rosai-Dorfman disease (RDD; also known as sinus histiocytosis with massive lymphadenopathy, SHML) was made. Increasing respiratory obstruction by lymphoid tissue prompted a 2-week trial with oral prednisolone. A dramatic response occurred, with complete resolution of all clinical findings within 5 days, but with recurrence of lymphadenopathy 6 weeks after stopping with medication. Further observations over the next 3 years established a consistent response to prednisolone and a pattern of steroid dependence. |
Databáze: | OpenAIRE |
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