Abstract

The co-occurrence of Langerhans cell histiocytosis (LCH; disorder characterized by proliferation of Langerhans cells) and Rosai-Dorfman disease (RDD; histiocytic entity that is one of the non-Langerhans cell diseases) is extremely rare and raises several questions regarding the nature of the diseases. We describe a 10-year-old boy who presented with a 3-month history of right scalp swelling. Clinical, pathological, and imaging evaluation disclosed multiple LCH of bone and cutaneous RDD. After initiating prednisone and vinblastine therapy, the patient developed an asymptomatic diffuse papular eruption. Biopsy revealed cutaneous RDD. Treatment was continued with a good response of bone LCH and significant amelioration of the cutaneous findings. Co-occurrence of LCH and RDD is a rare phenomenon. Various explanations, including the role of chemotherapy, are suggested based on our and several previously reported cases.

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