Histiocytosis X; Langerhans cell histiocytosis; Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis; Hand-Schuller-Christian disease; Letterer-Siwe disease
Histiocytosis X often affects the whole body. A disease that affects the whole body is called a systemic disorder.
Symptoms can vary between children and adults, although there can be some overlap. Tumors in weight-bearing bones, such as the legs or spine, may cause the bones to fracture without apparent reason.
Symptoms in children may include:
- Abdominal pain
- Bone pain (possibly)
- Delayed puberty
- Ear drainage that continues long-term
- Eyes that appear to stick out (protrude) more and more
Failure to thrive
- Frequent urination
- Mental deterioration
- Rash (
petechiaeor purpura) Seborrheic dermatitisof the scalp
- Short stature
- Swollen lymph glands
- Weight loss
Note: Children over 5 years old often have only bone involvement.
Symptoms in adults may include:
- Bone pain
Chest pain Cough Fever
- General discomfort, uneasiness, or ill feeling (
- Increased amount of urine
Shortness of breath
- Thirst and increased drinking of fluids
Signs and tests
The tumors produce a "punched-out" look on a bone x-ray. Specific tests vary depending on the age of the patient.
Tests in children may also include:
- Biopsy of skin to check for the presence of Langerhans cells
Bone marrow biopsyto check for the presence of Langerhans cells
- Complete blood count (
CBC) X-rays of all the bones in the body (skeletal survey)to find out how many bones are affected
Tests in adults may include:
Bronchoscopywith biopsy Chest x-ray Pulmonary function tests
Histiocytosis X is sometimes associated with cancer. CT scans and biopsy should be done to rule out possible cancer.