Steroid-Responsive Rosai-Dorfman Disease

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.