Histiocytosis

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Eosinophilic granuloma - X-ray of the skull

Respiratory system

Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis; Hand-Schuller-Christian disease; Letterer-Siwe disease; Langerhans cell histiocytosis

The symptoms that affect children and adults are listed below, although there can be some overlap.

Adult:

• Cough
• Shortness of breath
• Chest pain
• Fever
• Weight loss
• General discomfort, uneasiness, or ill feeling (malaise)
• Increased amount of urine output
• Thirst and increased drinking of fluids
• Bone pain
• Rash

Children:

• Failure to thrive
• Weight loss
• Irritability
• Fever
• Seborrheic dermatitis of the scalp
• Abdominal pain
• Jaundice
• Vomiting
• Limping
• Thirst and frequent urination
• Short stature
• Delayed puberty
• Mental deterioration
• Headache
• Dizziness
• Seizures
• Increased eyeball protrusion
• Swollen lymph glands
• Generalized rash (petechiae or purpura)
• Chronically draining ears
• Possible bone pain

Adult:

• Chest x-ray
• Bronchoscopy with biopsy showing typical abnormalities 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 how much of the skeleton is affected
• Biopsy of skin to check for the presence of Langerhans cells
• Bone marrow biopsy to check for the presence of Langerhans cells
• Complete blood count (CBC)
• Other tests as needed

Review Date: 10/30/2006
Reviewed By: William Matsui, MD, Assistant Professor of Oncology, Division of Hematologic Malignancies, The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, MD. Review provided by VeriMed Healthcare Network.