Rosai-Dorfman Disease

Rosai-Dorfman disease (RDD) is a rare disease characterized by proliferation of histiocytes in lymph nodes and other organs.  Skin involvement occurs in about 10% of systemic cases, but more cases of skin-limited RDD are being recognized, especially in women, based on recent case reports and personal experience.

The other name for RDD is “sinus histiocytosis with massive lymphadenopathy.”  The lymph nodes in the neck are most often enlarged on both sides in the systemic form.  Skin lesions may be solitary or multiple, papules or nodules, in hues of red, brown, and purple.  The face is commonly affected.  Blood abnormalities are also often seen in systemic RDD.

The cause of RDD is not known, but it is believed to be non-cancerous.   The diagnosis must be confirmed by a biopsy of a lymph node or skin lesion where the pathologic findings are distinctive and identical.  One unusual characteristic feature is emperiopolesis which means the engulfment of entire lymphocytes and plasma cells by the histiocytes.

While treatment is not always necessary, it may be indicated if lesions are particularly large, widespread, or destructive.  I have personally seen only two cases of RDD in over three decades of dermatology practice.  The first was a man with massive lymph nodes in the neck and disseminated skin nodules.  His physician was contemplating treating him with the anti-cancer drug Rituxan.  The second was a woman with a solitary nodule on the back.  We were able to completely excise that lesion surgically.

Two new cases of RDD limited to skin were published recently in Cutis (June and November 2012).  One patient was an elderly man with nodules on the left flank.  No treatment was rendered.  The other case report was of a 50-year-old woman with RDD limited to the face and back.  She was treated by liquid nitrogen cryosurgery with partial success after topical and intralesional corticosteroids failed.

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