The symptoms and physical findings associated with Rosai-Dorfman disease vary greatly from one person to another depending upon the extent of the disorder and the specific organ systems affected. Some cases may only affect the lymph nodes and may not cause any serious complications. Less often, some cases may affect various organ systems of the body and may potentially cause serious complications. Any organ system of the body may become affected.
In most cases, affected individuals exhibit painless Swelling or enlargement of affected lymph nodes (lymphadenopathy), most often those of the neck (cervical lymphadenopathy). Many individuals with Rosai-Dorfman disease do not develop any additional symptoms of the disorder (asymptomatic).
In some cases, affected individual may experience nonspecific symptoms that are common to many different conditions including fever, paleness of the skin (pallor), unintended weight loss, a general feeling of ill health (malaise) and a chronically runny nose (rhinitis). In extremely rare cases, affected individuals may experience abnormal enlargement of the liver and/or spleen (hepatosplenomegaly).
In approximately 43 percent of cases, other areas of the body besides the lymph nodes may also be affected (extranodal). Some individuals may have extranodal disease without the presence of lymphadenopathy. The skin is the most common extranodal site. Skin Lesions associated with Rosai-Dorfman disease are usually yellow or purple. A reddish rash-like Inflammation of the skin (erythema), small solid elevations on the skin (papules), or knots visible under the skin (nodules) may be present. Skin Lesions may occur anywhere on the body, but most often affect the head and neck. In some cases, Skin abnormalities precede the development of lymphadenopathy.
The salivary glands, nasal cavity, upper respiratory tract, various bones, and the eyes and eye sockets (orbits) may also be affected. In rare cases, the central nervous system, digestive system, or the kidneys may be affected. Involvement of these areas may result in additional (secondary) symptoms. For example, loss of vision may occur secondary to the involvement of the eyes, and Seizures may occur secondary to involvement of the central nervous system. In some cases of Rosai-Dorfman disease, the accumulation of histiocytes into masses may cause compression of vital organs potentially resulting in serious complications.
In some cases, the lymph nodes may not be affected. Instead a specific area of the body such as the skin, a solitary bone, or the central nervous system may be affected. These cases may be known as isolated Rosai-Dorfman disease. A distinct type of isolated Rosai-Dorfman disease has been identified called cutaneous Rosai-Dorfman disease, in which only the skin is affected. Initial reports indicate that cutaneous Rosai-Dorfman disease is more common in female adults.