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.
Antonius
J I
JI
Department of Pathology, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia.
Farid
S M
SM
Baez-Giangreco
A
A
D000284:Administration, Oral; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D015618:Histiocytosis, Sinus / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011239:Prednisolone / Q000008:administration & dosage*; D012208:Rhabdomyosarcoma / Q000175:diagnosis
D011239:Prednisolone