Rosai-Dorfman Disease


Symptoms

Rosai-Dorfman may affect lymph nodes only, it can affect lymph nodes plus other body systems, or it can occur in body systems without lymph node involvement. However, more than 87% of patients present with enlargement of the lymph nodes in the neck. This is usually on both sides and is painless but often get very big. Within the lymph node system, it can also occur in the nodes in the groin, armpits, and in the central part of the chest.

Location of this disease outside of the lymph nodes occurs in approximately 40% of cases. In order of frequency, this disease affects skin and soft tissue (16%), nasal cavity (16%), eyes/eyelids (11%), bones (11%), central nervous system (7%), salivary glands (7%), kidney (3%), airway (respiratory tract) 3%, and liver 1%. The breast, digestive tract, and heart are affected in less than 1% of cases.

Symptoms of RD depend on the site of involvement and can include:

  • Lymph node enlargement (with or without pain).
  • Fever.

More rarely

  • Weakness.
  • Paleness/anemia.
  • Weight loss.
  • Shortness of breath.
  • Blockage/discharge of the nose.
  • Nosebleeds.
  • Deformity of the nose (saddle-nose).
  • Inflammation of the tonsils/sinuses.
  • Difficulty swallowing or speaking.
  • High-pitched breathing.
  • Eye bulging/decreased vision.
  • Headaches.
  • Seizures.
  • Night sweats.
  • Decreased sensation.
  • Paralysis.
  • Blood abnormalities (low red blood cells, white cell abnormalities, increased sedimentation/inflammatory cell rate, abnormal protein levels, etc).
  • Joint pain.
  • Slowly growing, painless mass.

Many adults with RD experience severe and sometimes overwhelming pain associated with this disease. While pain can be caused by bone lesions or bone defects that do not heal completely with therapy, some patients have pain even when there is no active disease seen on x-ray. The cause of this pain is not understood; however, it is being currently explored in research. Pain is considered a complication of RD that should be fully evaluated by a physician and treated with appropriate medications.

It is important to remember that symptoms alone do not make the diagnosis of RD disease. Thus, it is important to consult a physician to receive a thorough workup and accurate diagnosis.

 

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