Histiocytosis

Alternative names:

eosinophilic granuloma; Hand-Schuller-Christian disease; histiocytosis X; Langerhans cell histiocytoses; Letterer-Siwe disease; nonlipid reticuloendotheliosis; pulmonary histiocytosis X; pulmonary langerhans cell granulomatosis

Symptoms:

Adult:

• cough
• shortness of breath
• chest pain
• fever
• weight loss
• general discomfort, uneasiness, or ill feeling (malaise)

Children

• failure to thrive
• weight loss
• irritability
• fever
• seborrheic dermatitis of the scalp
• generalized rash (petechiae or purpura)
• chronically draining ears
• bone pain may or may not be present

Signs and tests:

• chest X-ray
• bronchoscopy with biopsy showing typical X bodies in histiocytes
• pulmonary function tests

Children:

• bone X-ray showing punched-out appearance of bone lesions
• skeletal survey X-ray (X-rays of the entire skeleton) to determine the degree of bone involvement
• biopsy of bone to check for the presence of Langerhans cells
• biopsy of skin to check for the presence of Langerhans cells
• bone marrow biopsy
• CBC