فهرست نشریات علمی دسترسی آزاد ایران

Rosai-Dorfman disease (RDD) is a rare, benign, and idiopathic condition characterized by the abnormal proliferation of histiocytes, also known as sinus histiocytosis with massive lymphadenopathy. About 40% of cases exhibit extranodal manifestations, with the skin being one of the most commonly affected sites. The disease has distinct histopathological features, including emperipolesis and the accumulation of CD68-positive, S100-positive, and CD1a-negative histiocytes. We report the case of a 49-year-old woman who developed red-brown, dome-shaped lumps on her upper arm and thigh that progressively enlarged over time. Dermoscopy revealed milky-red areas with multiple irregular yellowish homogeneous structures on an erythematous-orange background, along with telangiectatic vessels. Ultrasound revealed the presence of multiple lymphadenopathies. The patient underwent several biopsies; the most recent biopsy demonstrated characteristic features of Rosai-Dorfman disease, specifically emperipolesis and positivity for S100 and CD68 on immunohistochemistry. Treatment with intralesional steroids and fenofibrate led to improvement of the skin lesions. The rarity and diverse nature of Rosai-Dorfman disease present significant challenges in its diagnosis and management. A comprehensive evaluation and a multidisciplinary approach are essential for effective treatment of this condition.