Rosai-Dorfman disease also known as sinus histiocytosis with massive lymphadenopathy (SHML) is characterized by distorted lymph node architecture with marked dilation of lymphatic sinuses occupied by numerous lymphocytes, as well as histiocytes with vesicular nucleus and abundant clear cytoplasm with phagocytized lymphocytes or plasma cells, also known as 'emperipolesis'. This disease of unknow...

BACKGROUND Rosai-Dorfman disease is a non-Langerhans cell histiocytosis that recently has been treated successfully with imatinib mesylate in a patient with a systemic variant of the disease. OBSERVATIONS We describe a 69-year-old man with cutaneous Rosai-Dorfman disease manifesting as progressive, deeply infiltrated skin lesions. Histopathologic examination of the lesions demonstrated dense ...

OBJECTIVES Programmed death-1 (PD-1) is physiologically expressed by germinal center (GC)-associated helper T cells. It has been proposed that an increase in PD-1+ cells outside GC could indicate pattern I angioimmunoblastic T-cell lymphoma (AITL). METHODS We studied the distribution of PD-1+ cells in reactive lymphadenopathies (LA), including HIV-associated and dermatopathic LA (n = 5, each)...

An erythematous cutaneous plaque on the breast with no signs of infection requires a skin biopsy to rule out dermal extension of an underlying mammary adenocarcinoma, primary skin tumour (e.g. angiosarcoma or malignant lymphoma) (1), or inflammatory lymphoedema of the breast (2). It can more rarely reveal location on the skin of systemic disease. Rosai-Dorfman disease (RDD), also known as sinus...

We report a 48-year-old man who presents with a 20-month history of left parotid enlargement despite treatment with antibiotics and steroids. He presented with a non-painful palpable mass in his left parotid without facial weakness or otologic symptoms. Fine needle aspiration was inconclusive, and an attempted excisional biopsy was aborted due to involvement of the facial nerve and suspicion of...

A young male presented with recurrent neck swellings with initial leucocyte count of 16800/mm3, with non-caseating glands on ultrasonography neck. FNAC showed mixed cellularity with histiocytic and marked lymphophagocytosis as seen in Rosai Dorfman Disease. Immunohistochemistry demonstrated CD 25, Ki 67 and CD 68 in histiocytes. Her responded to supportive treatment alone.