I can't seem to find much on this except for a message forum, can anyone tell me about this disease....the symptoms, treatment, etc...my best friens son has just been tested for this yesterday and they won't know anything for a few days....All I know is that he had some sort of lesion or small lump on his skull and the hair on that area fell out. Thanks.

From Harrison's Principles of Internal Medicine, 14th Ed. (McGraw Hill, 1998):

Histiocytosis X is also known as:
- Langerhans Cell Granulomatosis
- Eosinophilic Granuloma

Classified as an interstitial lung disease (ILD).

quote:

---

This condition is being recognized with increasing frequency and may account for about 1 to 5 percent of ILD of unknown etiology...The pulmonary form of this disease occurs in young and middle-aged adults, usually males, and in those who use tobacco heavily; it may remain focal or involve one or several bony sites. Occasionally, multifocal disease can affect the posterior pituitary gland, causing diabetes insipidus, a condition that is termed Hand-Schuller-Christian disease. In infants, Letterer-Siwe disease is a more fulminant visceral form of this disorder that mimics a malignant lymphoma. In adults the presenting symptoms and signs do not distinguish this disease from other forms of ILD unless signs of a bone lesion exist...As the disease progresses, greater airway obstruction may develop, and the chest radiograph can resemble that in advanced chronic obstructive lung disease...[Treatment] involves a mandatory cessation of tobacco use, which may cause the pulmonary disease to stabilize or regress. Glucocorticoids usually are not helpful. Penicillamine has been used in an attempt to prevent fibrosis, with variable success. Local bone lesions may require irradiation treatment. For patients with increasing symptoms of airway obstruction, supportive therapy and bronchodilators may be tried, but their success has been modest. Lung transplantation should be considered for certain patients.

---

I skipped over technical stuff about the molecular biology and microscopic findings. For a book of this size (over 2500 pages of fine print) there is remarkably little written about the disease (less than half a page).

Wish there was better news. Maybe they should seek a 2nd opinion and get referred to a good pulmonologist. Hope any of this helps.

Thanks professor, they did take him to Childrens Hospital and will most likely seek a second oppinion IF they can, they are really strapped for cah and have no health insurance right now. I will just pary for good news. Thank you again.