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.

Typical skin nodules seen in Rosai-Dorfman Disease

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.

Other Interesting Case Studies:
How Efficient Is Your Dermatologist? Example from My Practice-Eczema or Ringworm
Painful Oral Erosions and White Patches

Tagged , , , , , , . Bookmark the permalink.

4 Responses to Rosai-Dorfman Disease

  1. Eric Pan says:

    Hello Doctor Charles:

    We are from China, and my father suffered the RDD from his nose, but now the tumour already go into his brain. With the surgery did last year, our doctor told us they already took off almost 90% of the tumour, but unfortunately, my father’s eye is oppressed by the tumour. We are in China, and looks our doctors in China have no idea for RDD. Would you plz kindly suggest us some idea for his disease? And looking forward your reply and let me know which information you need?

    • Heather Mason says:

      My daughter’s situation is the same as Eric Pan’s father, but she is just 14 years old. I am very interested to know what happened in his case, please advise.
      Specifically my daughter had surgery on one eye, and the pathology determined it was Rosai Dorfman’s, on her other eye the doctor’s are extremely leery to perform surgery as the tumor is between her eye and brain. We have been through 6 months of chemotherapy but there has been little change. We are looking for options.

  2. Lineice Davis says:

    I have a twelve year old daughter who has been fighting this for a few years now.She’s have had two eye surgery’s,nose and she’s got one on her aorta on her heart. All they can say is they are going to watch it.

    • Dr. Camisa says:

      I am sorry to hear this. It is very unusual for children to have R-D disease. My own practice is limited to adults only. I would suggest that you obtain a second opinion from pediatric oncology at a major academic center such as Mayo Clinic (Rochester,MN) or MD Anderson (Houston,TX). Get a copy of all of her records to bring to this consultation so they don’t have to repeat so many tests.

Leave a Reply

Your email address will not be published. Required fields are marked *