Steroid-Responsive Rosai-Dorfman Disease
- 1 January 1996
- journal article
- case report
- Published by Taylor & Francis Ltd in Pediatric Hematology and Oncology
- Vol. 13 (6), 563-570
- https://doi.org/10.3109/08880019609030873
Abstract
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.Keywords
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