Nearly any part of the body can be involved with LCH, though some sites are more common than others. A patient may have very limited involvement in only one body system or widespread involvement in several different sites and systems. Some of the body systems that may be affected by LCH in adults include the following:

• Skin (rashes or ulceration). Skin involvement is very common in adults with LCH and may include areas such as the scalp, face, anal area, vulva, and flexural areas of skin such as the groin, under the breasts, on the neck, under the arms, and behind the ears. Skin over involved lymph nodes or bones may also be affected and sometimes even the nails. Typical lesions of the skin may include small solid reddish elevations on the skin surface, knots under the skin, purplish-red spots, bleeding under the skin, rashes that are scaly and greasy, ulcerations, and small abscesses. Since many skin problems often are cause by dysfunction of other body systems, sometimes the type of skin lesions a patient has may signal that other areas in the patient are affected.

• Bone (single or multiple lesions). Bone lesions, another very common occurrence in adults with LCH, may cause pain, swelling, spontaneous fracture, or ulceration at the site, or they may cause no symptoms at all before detection. Although nearly any bone can be affected, skeletal involvement most commonly includes the skull, other flat bones such as the ribs, and long bones such as the arms and legs.

• Lung (dysfunction). The lungs are frequently affected in adults with LCH. As a matter of fact, some adults are diagnosed with primary eosinophilic granuloma (also called pulmonary Langerhans cell histiocytosis or Langerhans cell granulomatosis). Recent research suggests that there is a connection to smoking in this isolated form of LCH since some studies have shown that a significant percentage of patients with this form are smokers. This form may go into remission when the patient stops smoking. More often, however, lung involvement occurs with more widespread LCH and sometimes progresses to lung failure. The symptoms of lung involvement include shortness of breath, chest pain, dry cough, and in extreme cases, lung collapse. However, in many cases it may show no symptoms at all before being detected accidentally on a routine chest x-ray.

• Teeth/Gums (loose/lost teeth, swollen gums, ulcerations). The teeth and gums are often affected due to extensions of the disease from the jawbone to the structures and interior coverings of the mouth. Mouth ulcers may be mistaken for canker sores.

• Ears (chronic ear infections and/or discharge). Ear involvement, often a result of skull lesions expanding to the ear, may cause chronic drainage, ulceration, and balance problems.

• Endocrine System (pituitary gland and, rarely, the thyroid gland). Involvement of the pituitary gland can cause excessive thirst and urination, signs of a condition known as diabetes insipidus. A special test, called a water deprivation test, can be preformed to diagnose this condition. Although there is no cure for diabetes insipidus, it can be controlled with the use of a synthetic hormone. Other hormonal abnormalities caused by thyroid or pituitary involvement may result in menstrual problems for women and weight gain or loss.

• Female Genital/Reproductive Tract (vulva, vagina, and ovaries). Most commonly, LCH in this area causes inflammation, rash, and/or ulceration of the vulva, vagina, and/or cervix, but it may also affect the ovaries, causing dysfunction and possibly failure of these organs.

• Lymphoreticular System (dysfunction of liver, spleen, lymph nodes). These areas of the body are less commonly involved in adults with LCH, but when affected, patients may experience enlarged lymph nodes, spleen and/or liver, which may result in fatigue and other illnesses or complications associated with dysfunction or failure of these organs.
  
  
• Gastrointestinal System (problems with stomach or intestines). Involvement in this body system is very rare in adults, but symptoms might include diarrhea, nausea, vomiting, and/or weight loss.
  
  
• Central Nervous System (spinal cord and brain lesions). Another rarely affected body system is the central nervous system. Symptoms of CNS involvement may include staggering when walking, seizures, weakness of the arms, legs, or weakness of one side of the body. Since the pituitary gland could be affected, lesions of this system may also cause some of the same endocrine problems as mentioned above.

• Other Symptoms. Other general symptoms which may be difficult to connect specifically to one of the systems above include fever, weakness, fatigue, and sometimes weight loss.

It is important to remember that patients may not all have all the areas of involvement mentioned above, nor would it be accurate to say that someone with only one system involved will not go on to develop problems in other systems. These are some of the many uncertainties surrounding this disease, and it is these uncertainties at diagnosis, which can be extremely frustrating for both the patient and the physician.